Biochemistry Mega Revision - Part 1

hello everyone happy evening a very good evening I hope and I believe all of you are doing well a quick note whether the audio visual is all good let me just check the live chat all right I believe or you can uh see me and hear me as well so here we are in the biochemistry Mega revision uh where we would try to revise uh all the important points and the must know points in Biochemistry and this is the first part itself this is the part one itself that we are doing here today right okay and apart from that we have a few other apart from that we have other free live classes also going on so on the unacademy app at 6 PM starting today we have planned for three classes uh so 6 pm today we had the class on physiology graphs and few numericals uh tomorrow 6 PM we will have a class on PSM numericals basically telling you the tricks on how to solve the questions fast and the mnemonics to remember the various the p-value sensitivity specificity all of that and on 26 it would be basically pre and para clinical subjects the clinical way there would be kbmd of that uh so clinical questions of first and second year uh second year subjects is what we are going to do I've shared the links on the telegram group already we will have part two of the biochemistry Mega revision tomorrow at 9 30 PM okay uh yes thank you so much all these classes are planned because based on the feedback the inputs that I've received from majority of you so thank you K and everyone else here a quick update on what's happening on the platform so the unlock is back uh we you have now uh 20 percent of available on medical PG subscriptions so fmg students can take for the fmg Mastery course we have for the next students as well and you have the code Dr Nikita for the 20 off you also have now the short shot 150 available at a very very reasonable price of 1600 with the credits that you have on the app you can get it for as low as 1200 so this is still uh 28 February we have this ongoing offer in the Aspire next 2023 batch uh basically it's a nine months course we are starting on February 28th we have a really good Educators to cater to your subjects it's gonna cover the theory the mcqs the clinical mcqs and everything for the current exam going students we have the final booster batch a one month rapid revision course now available at 4K so pre by queues most expected questions VTR is available in this final booster batch and for the fmd students we have started the batch on February 10th by April we will be finishing the theory and then in the rest of the two months we would be focusing on the McQ practice right so let us start with the first one so I'll be first starting with the topic of amino acids because this is a very very important and first we'll do the structure part of it then the amino acid disorders expect one question from that right so amino acid special groups so I'll be quickly telling you everything because there are so many things to be revised so we'll go a bit fast you can always come back to this video later and you can revise as well later on as well okay so remember that guanidinium group guanidinium G4 G is present in arginine Benzene group is present in phenylalanine when I say phenyl we know that it has that Benzene ring and tyrosine has a phenol group okay five tyrosine has a phenol group histidine is imidazole so remember it is high high means histidine has imidazole group Proline is pyrolidine Ryman Proline and pyrolidine methion and thiothio is The thio Ether linkage and tryptophan has a indole group so remember this tryptophan is like try the tri-color flag of India so India is indole and this is tryptophan so remember tryptophan has indole group and also remember that tryptophan try for vitamin B3 it forms vitamin B3 60 milligram of cryptofan forms 1 milligram of acid right so that is why whenever there's going to be a tryptophan deficiency there's going to be niacin deficiency like you have in conditions like hot laps carcinoid where all the tryptophan is going towards serotonin okay next one uh so which amino acids are most acidic and which are the most basic so thinking logically acidic acidic aspartic acid glutamic acid or acidic aspartic acid a a is very very acidic okay and the next that we have is a basic basic basically means Alkali you can remember it as Alkali Alkali is h a l so we have histidine Arginine and lysine which are the most basic arginine is the most basic so remember that a a are the ones which are the most acidic and most basic so if I tell you which is the most acidic aspartic acid which is the most basic again a for arginine okay a for arginine so remember Alkali is alkaline that is histidine Arginine and lysine Are alkaline that is basic now which are the essential amino acids and which are the non-essential amino acids for the essential amino acids we have um private Tim Hall okay few queries here what is the duration of this particular class this is going to be around 50 minutes one hour class part one next will be part two tomorrow is this enough for the last minute revision I would not say this is enough but this is the core this is like must know you cannot go to the exam Hall without knowing this I hope this answers all the queries that I've been getting here in the live chat okay so private Tim Hall is the essential amino acids P for phenylalanine valine and remember T is three on in okay T is three on it it is not tyrosine uh I'm really sorry could not share the PDF before the class should have done it but I forgot I'll do it after the class is over okay then you have t for tryptophan isoleucine methional histidine Arginine and Lucin and lysine some books also put h and a it is into a semi-essential so you can remember ah it comes as a surprise these are not essential these are like semi-essential so Arginine and histidine are some books uh put it into uh semi-essential now the next one that we have is so essential and semi-essential we know now now tell me that which are the amino acids which are both uh glucogenic and ketogenic amino acids which are both glucogenic and ketogenic are remember this is a tip to remember that which are both glucogenic and ketogenic it is tip what does tip stand for tryptophan isoleucin phenylalanide and the other t for tyrosine so basically all the aromatic amino acids phenylalanine tryptophan tyrosine and isolucine you can remember that I saw Lucin ISO Matlab acting or present in isoform that is two forms that is glucogenic and ketogenic so remember aromatic amino acids plus isoleucine these are both glucogenic and ketogenic also remember that which are the amino acids which absorb of the ultraviolet light those are the aromatic amino acids phenylalanine tyrosine tryptophan maximum is with tryptophan okay maximum is with tryptophan this is a previously asked question that if you replace ln9 with which amino acid then there will be light absorption at 280 nanometers that is tryptophan next one which are the pure ketogenic amino acids remember L and L okay L and L then what do we have is ketogenic so remember ketogenic is leucine and lysine and all the rest of them they go into glycogenic so remember which is ketogenic remember which is both if not any of that then that amino acid is glucogenic then that amino acid is glucogenic okay now going to which are the branched chain amino acids which are the branch chain amino acids remember a tree which is branching right a tree which is branching which is that tree this is a maple tree which has the branches right so why am I telling you maple tree because the defect in the branch in amino acid metabolism leads to maple syrup urine disease when do we Branch out into the multiple Generations right we Branch out into multiple Generations when we are live when we live when we are live so remember l i v what is l i v l i v is Lucin isolucin and valine remember L is not for lysine okay L is not for lysine so when I say isolution it is not lysine it is not lysine right out of that we saw that isolution is both is only ketogenic and valine is glucogenic right so you can also be asked a question like which of these is a branch chain amino acid and is both leukogenic and ketogenic you have the options remember it is isoleucine okay it is isoleucine so maple syrup urine disease is a defect in Branch chain ketoacid dehydrogenase one two three four letters one two three four letters voila enzyme msud is bckd and remember that What vitamin supplementation can help in msud it is vitamin B1 thymine is a very important part of bckd this bckd requires what other vitamins one plus two three two plus three five one plus two three two plus three five vitamin B1 B2 B3 B5 and it requires lipoic acid okay so vitamin B1 supplementation is what we give uh next we have is enzymes what are the various enzymes defective in the various amino acid disorders so what do we have phenyl Ketone urea as the term says it is phenylalanine hydroxylase phenylalanine hydroxylase deficiency tyrosinemia type 1 which is tyrosynosis remember one is first first is fumeral acetoacetate hydrolase that is f a a h maple syrup maple tree it is branching Branch chain Alpha keto acid dehydrogenase Al caponeuria is homogenicid epoxy days okay homogenicide oxidase alcaptainuria is a defect in which amino acid metabolism while Captain urea is a defect in tyrosine metabolism okay it's a defectant tyrosine metabolism albinism again is tyrosinase because milanin comes from tyrosine homocysteine urea type 1 that's a classical one so remember that homocysteine is Sister thionine synthesis which is What vitamin dependent homocysteine is like homo 16 so remember it is vitamin B6 this is dependent on vitamin B6 uh homocysteine urea type 2 is the other one that is B12 deficiency then you have tyrosinemia type 2 which is Rich norat syndrome two has two words richner hanat and remember 240 and the enzyme is that that is tyrosine amino transferase or tyrosine trans aminase tyrosine Amino transferase okay and when in which amino acid metabolism defect do we see hyperoxylurea remember glyoxylate that is basically glycine glycine metabolism defect May hyperoxyl urea okay remember that now what are the tyrosinemia types you have type 1 type 2 type 3 and you have your Hawkins urea type 1 is tyrosynosis this is the one which is called as hepato renal this is the one which increases the risk of HCC and F for first f a a h is the enzyme tyrosinemia type 2 that is richness syndrome is that this is what we already saw this has oculocutaneous involvement and which is the neonatal tyrosinemia that is tyrosinemia Type 3 which can be seen as a benign condition in the newborn because of the functional uh you know the function is less developed in the new bonds for the enzyme for hppd hydroxyphenyl pyruvate dioxygenase so remember in type three three four it is 4 hppd which is defective if it is a mutation in the gene for hppd remember h4h then it leads to Hawkins urea okay then it leads to Hawkins urea so remember one is first faah2 is that 3 is 4 4 hppd one is the one uh which is the most severe and it has what order it has cabbage odor it increases the risk of HCC remember cab car tire that means cabbage odor is seen in tyrosinemia and from the tire fumes are coming out that means tyrosinemia forms fumerate as the product you have fumaril acetoacetate hydros uh hydrolase next one looking at the urine order urine order my maple syrup means Maple syrupoga remember syrup is the sugar that means it has burnt sugar order okay it has burnt sugar order which is also called as caramelized sugar it means the same thing syrup contains sugar so maple syrup is burnt sugar Hawkins remember Hawk is a bird who is swimming in the swimming pool so Hawkins is swimming pool uh then you have sweaty feet sweaty feet is isovaloric when I write ISO valeric remember the SV is the sweat sweaty feet cabbage is tyrosinemia fumes multiple carboxylase deficiency this is the c a m c a m c a this is Tom cat urine so remember tom cat urine is multiple carboxylase deficiency phenyl phenyl is like f f is for M M is for f phenylketonuria is mousy order methylamineuria is fishy odor this is a previously asked question in one of the recent fmge exams okay so remember methylamine urea is uh is a rotting fish uh these are the ones that we have already covered here the odor in the various Okay order in the various amino acid disorders next one homocysteine urea is something which is very very important and remember few points about homocysteine urea clinical features are this patient habitus is similar to marfons that means this is a tall patient with the long fingers and what else do we have the keywords are early age am I early age stroke because homocysteine urea is like a pro atherosclerotic Cloud condition okay it fastens the atherosclerosis like Marfan this also has the lens subluxation but where do you have the lens of luxation and homocysteinuria it is opposite of marfons where do you have in marfons remember marfons is like Mar fan is present up in the ceiling and we say everybody fan out so in Marfan it is up and out so in homosystem area it is down and in so it is down and in that means it is infero medial okay that means it is inferometer inferonasal is what you should remember right so and here the IQ also can be affected in homocysteine urea so few questions on homocysteineuria homocysteineuria is formed from which amino acid homocysteine urea this is basically a reversible reaction that is present here methion informs homocysteine homocysteine forms methionine so remember homocysteine is formed from methionine next Once the homocysteine is formed either it can go to form methionine back to methionine or it can go ahead and it can form cysteine right so remember it is m h c like your major histocompatibility complex H can form m h can form C that means it can form methional or it can form cysteine and of to form the cysteine this is vitamin B6 dependent so homocysteine is basically homo 16 getting converted to cysteine that is again like 16. this requires vitamin B6 okay requires vitamin B6 so sister thionine synthesis is vitamin B6 right so when there is homocysteine urea this step is blocked and that is why homocysteine gets elevated so therefore if you get a question that in homocysteine urea uh what what should be supplemented and what should be stopped remember that is supplementation needs to be given methionine should be restricted because methionine forms homocysteine we do not want more homocysteine so restrict methionine gives cysteine in homocysteine urea okay and for the conversion of homocysteine to mithyan so this requires methyl Cobalt a mind that means vitamin B12 and vitamin B12 bhakti the post test is at 10 30 I believe right it's not 10 I'm sorry okay so what do you need to remember is vitamin B12 is required for conversion of homocysteine to methion and methyl Cobalt amide and b12 and B9 that is B12 and folic acid are very interrelated right so remember this is B9 and b12 So What vitamin deficiencies basically can lead to homocysteine urea remember the multiples of three but not starting with three that is 6 9 12 vitamin B6 vitamin B9 vitamin B12 but not vitamin B3 niacin deficiency will not cause homocysteine urea okay 6 9 12 6 is the classical one okay six is the classical one if you have to choose one so homocysteine urea intellectual disability stroke Mi marfelloid habitus lends the luxation which is infero medial kyphosis skeletal formation deformities and osteoporosis decrease methion increase the cysteine you have B6 B9 and b12 supplementation is what we need okay and this is what is given here basically TK next one let's go to phenylalanine metabolism now phenyl Allen 9 it forms tyrosine by phenylalanine hydroxylase so that is why when this enzyme is deficient this leads to phenyl Ketone urea so there would be phenylbutyrate of phenyl lactate that would be formed and so there will be phenylketone which will increase which gives the mousy odor or the musty odor okay it gives the mousy or the musty odor what are the clinical features in phenyl ketoneuria remember that the clinical features are blonde hair is a feature given with a mental retardation IQ is decreased okay another question if the mother has phenyl Ketone urea during pregnancy that means what is the teratogenic effect on the child it can lead to microcephaly okay it can lead to microcephaly next one that we have what is the other thing that this phenylalanine hydroxylase requires it is a tetrahydrobiopterin that is bh4 tetrahydro B for biopterin is required okay so remember that can lead to non-classical phenyl ketoneuria so tyrosine tyrosine so what amino acid becomes essential in a phenyl ketoneuria it is tyrosine normally tyrosine can be formed in the body from phenylalanine but if there is a deficiency at this step tyrosine will not be formed so tyrosine will become essential next one that we have is uh tyrosine goes ahead and forms dopa dopa forms dopamine norepinephrine epinephrine so basically the catechol amines all of these are formed from tyrosine you can remember this as tyrosine sounds similar to thyroxine so when thyroxine is increased in the body hyperthyroidism we know that there is a lot of catecholamine reaction sensitivity there so remember that thyroxyl and catecholamines go together and both of them are formed from tyrosine so a tyrosine by tyrosine hydroxylase forms dopa even the styrosine hydroxylase requires tetrahydrobiopterin so all the three aromatic amino acids hydroxylase phenylalanine hydroxylase tyrosine hydroxylase tryptophan hydroxylase and you have nitric oxide synthase these require tetrahydrobiopter and what is the reaction which basically converts a norepinephrine to epinephrine it is a methyl transferase that is methylation so remember that norepinephrine has no methyl group when you add a methyl group then it becomes epinephrine which is a methyl donor in the body it is it is s adenosyl methionine that is Sam you can see that methion and here is forming homocysteine as adenosine homocysteine so methionine is forming homocysteine okay dopamine forms norepinephrine by hydroxylase dopamine hydroxylase What vitamin is required for hydroxylases hydroxy is water where is the water present water is present in the sea so remember this requires vitamin C hydroxylation dopa forms dopamine by dopa B carboxylase so always remember this point that whenever an amino acid is forming an amine amino acid forming an amine that means you are removing the acid group so it is the carboxylation reaction this requires vitamin B6 okay this requires vitamin B6 the carboxylase requires vitamin B6 okay uh so now going to the next one so phenylalan9 uh tyrosine this can lead to phenylketon urea if this is stopped tyrosine forms dopa from dopa you have Milan and also by the action of tyrosinase so albinism is tyrosine deficiency remember albinism melanin is formed from tyrosine so it is albinism from tyrosinees dopa to dopamine we said it is vitamin B6 dopamine to norepinephrine vitamin C hydroxylase this is methylation this is Sam even homogenic acid comes from tyrosine that is why Al captonuria homogeneity oxidase deficiency is tyrosine deficiency key word for Al Capone urea remember Alka just rearrange that it is kala so you get kala urine on standing black urine on standing with El capto Nuria with pigmentation of the cartilage with okronosis and the patient is at risk of developing arthropathies early age arthropathies with Al captonuria remember it is autosomal recessive majority of these amino acid disorders are autosomal recessive okay they are autosomal recessive right going on to the next one this we have already seen phenylketonuria so decrease phenylalani because it is not getting metabolized so no need of giving that increase the tyrosine in the diet give bh4 supplementation Fair complexion mousy odor intellectual disability is what we see the phenylketones will increase in the urine okay next one uh next one this is what you are seeing here the same thing has been shown here remember that tyrosine forms Fumarate and acetoacetate types we have already seen going to amino acid products amino acid products quickly revising this is the same thing which is given here this we have already seen Millennial and thyroxine is also from tyrosine remember that what does tryptophan form trypto is tried to Tri is vitamin B3 and trypto is to serotonin and melatonin okay remember not milanin melanin is from tyrosine right absolutely right tire is black in color so remember black is melanin serotonin melatonin comes from cryptophan STD forms carnosine histamine is from histidine by which reaction you are forming the amine so this is decarboxylation okay glycine is required for a lot of things most important remember it's required for hemosynthesis and many other things is where we would see that and glutamate is G4 Gaba glutathione okay glutathione also requires glutamate very very important you'll see this question very frequently in the exam nitric oxide synthesis requires Arginine that is nitric oxide okay nitric oxide arginine is required for urea also and creatine also okay urea and creatine also so remember that if you get a question that arginine is required for synthesis of creatine urea nitric oxide all of the above remember it is all of the above okay do not be in a hurry to Mark the answer key Arginine in a nitric oxide always take the last option if you have all of the above okay going on to the next one so a quick revision of the products melanin is from tyrosine melatonin is tryptophan t3t4 thyroxine is again tyrosine histamine is from histidine Gaba is from glutamide B3 tryptophan creatine requires what all amino acids create always try to create a magnificent results mag are the amino acids required so you have methionine Arginine and glycine what are the three amino acids methionine Arginine and glycine are required then serotonin is cryptophan tytical amines thyroxine Vala that means it comes from tyrosine okay it comes from tyrosine the next one creatine synthesis what are the steps in creatine synthesis where does it occur first step is in the muscle and the liver or in the kidney first step is in the muscle kidney or liver where is the first step for creatine synthesis it's in the liver everybody's saying liver the steps of creatinine synthesis the first step is in the kidney then in the liver and then in the muscle easy to remember alphabetically remember h i j k l m so kidney liver muscle so first it starts with the kidney the first thing to form in the kidney is what is getting formed is guanidino acetate guanidino acetate guanidinium containing amino acid was Arginine so this requires Arginine and glycine okay so remember it is KLM is what you need to remember so guys look at the amino acids required for creatine you have Arginine Glycine and methionine that is mag result okay that is mag result uh next one what do we have is a glutathione glutathione what does glutathione require what does the glutathione require which amino acids remember glutathione is three that is g c g glutamate glutamic acid is required thiocontaining cysteine is a sulf hydro group containing thiocontaining and again you have glycine here okay again you have glycine here so look at this one uh this is glycine assistine and glutamate that is g c g that is for glutathione and which is the most functional the most active amino acid here for reduced glutathione the one which contains the sulfhydryl group reduce the glutathione is the cysteine cystinavala is the most active Okay cysteine is the most functional site there purine and pyrimidines what do they require remember that purines are two rings they have two rings pyramiding have a single ring okay pyramidine have a single ring so purines are two rings so they require more amino acids as compared to pyrimidines so Puri imagine like which are the purines those are the A and the g adenine and guanine these are the purines right and then you have when you have egg puree someone who has not had egg anytime has an egg puree gets a gag reflex so remember gag are the amino acids required you have glutamine Glycine and aspartate that is g a g okay that is g a g is required for purine in pyramidi we do not require pyrimidine so one amino acid is removed from gag the G is removed that is the glycine is not required you have glutamine and aspartate remember it is glutamine and aspartate not aspar Gene and glutamate it is glutamine and asparted which is required for purine and pyrimidine next one coming to a glycogen storage disorders are very very important so we have seen about the amino acid disorders very very important phenylketonuria El captonuria albinism then you have maple syrup urine disease a question on this uh generally comes in the exam very frequently next one glycogen storage disorders glycogen storage disorders quick points type 1 is one gurkis okay type one is one agar cheese and G 4G gurkis is glucose six phosphatase is the enzyme deficient so one gurkis is glucosa six phosphatase can you tell me this is a previously asked question glucose 6-phosphatase is a marker of which cell organelle glucose 6-phosphatase is a marker of which cell organelle it is a marker of endoplasmic reticulum okay it's a marker of endoplasmic reticulum very good so in what the key is the glucose 6-phosphatase is gone and this glucose 6-phosphatase what is it required for is it required for glycogenolysis is it required for gluconeogenesis or this is required for both remember this is required for both okay it's an enzyme required for both so when glucose 6-phosphatase is not there that is one gurkis both the glucose production pathway will be affected in the body and that is why there will be severe fasting hypoglycemia which will not be responsive even to glucagon challenge test will not be able to increase the glucose levels Plus everywhere the glycogen is deposited so there is hepatomegaly that is renomegaly there is hyperlipidemia so fat deposited in the cheeks imagine like chubby chicks it is called as doll like faces dog like fishies is another important feature as we have lactic acidosis and hyperuricemia okay these are very very important features of one gurkis okay yes that was the trick that we had posted on Instagram today itself in the 50 day challenge Victorious people can always make history today okay so one garkis pumpes curries Andersons mercado's hers and taruis these are the glycogen storage disorders one by one Victorious people can always make history today okay they can always make history today also okay then you have type 1B where is there is the transporter defect only okay the transporter Defector only this will have recurrent infections also type 2 is uh pumpies okay type 2 is pumpies Victorious People pumpies affects the pump in the body that is the heart so that is why there will be cardiomegali and heart failure heart requires fatty acid right so remember that the enzyme which is defective here is acid maltase acid maltase which is also your lysosomal enzyme so pompase is the only one which is a pump is the only one which is the glycogen storage disorder which is a lysosomal storage Disorder so it is actually Alpha glucosidase okay this is Alpha glucosidase enzyme okay next one type 3 the third alphabet is C this is Corey's also called as Forbes or limit dextrinosis has a defect of a d branching enzyme okay remember here also there is muscle weakness uh there is hepatomegaly due to accumulation of branched chain polysaccharide because the D branching is not happening so you will see the short branches which will be present that will lead to hepatomegaly Type 4 remember when I write 4 and I make it like a 4 is like a so that is Type 4 is under sensor disease remember Anderson dies at the sun's age that means that is at five years young age because of there is liver cirrhosis remember there is cirrhosis in Andersons okay and here there is a problem with branching Anderson branching Corey's D branching so what happens in Anderson's you would see long polysaccharide chain without branches that will accumulate so this will precipitate in the liver and that will cause cirrhosis so remember a for a this is also called as a myelopectinosis because it is like amylopectin the long chain type of 5 is the MAC address is the muscle glycogen phosphorylase hers is the hepatic glycogen phosphorylase mccardless muscle hers hepatic okay these are type 5 and type 6. muscle is gone so exercise induced cramps muscle breakdown okay Rhabdomyolysis myoglobin urea the renal failure yoga and very important remember there is a second wind phenomenon okay Second Wind phenomenal that means after this you know a break in the exercise the person can exercise again it's because of the increased cardiac output The increased blood flow to the muscles that's a compensatory mechanism so remember second when the phenomenon is seen in mcardle's disease WKA Ulta is m when you have uh the liver hepatic it's a mild hypoglycemia versus one gurkis which has severe hypoglycemia type 7 is taruis remember when I write T it has seven in it so remember taru is a seven and taruis is like tone rbcs so there is hemolytic anemia in taruese whenever the rbcs are affected uh the cycle that we are thinking about is glycolysis so glycolysis enzyme it is phosphofructokinase so this affects the muscle phosphofructokinase so exercise Wale cramps or hemolytic anemia is what we would see here so that is phosphofructokinase affected so these are the glycogen storage disorders have a look at this image also given in your first aid so what happens here is uh look at the first one what do you have at the first one the second is glycogen synthase this is how the glycogen is formed then occurs the branching okay then occurs the branching basically right and then what happens this one which you see where the glycogen can be uh degraded breakdown in the lysosome this is going to be pumpies okay this is going to be pompies then the next that we have which is glycogen phosphorylance defective it is a MAC address and where you have the short branches which are not able to be removed this is chorized the D branching is affected okay the D branching is affected right hurlers and Hunters remember these come under mucopolysaccharidosis hurlers Hunters her loss is l i duronides Hunters is ideuronate sulfur taste TNT L and L and in both of them it is heparent sulfate and dermatin sulfate which accumulate so in hurlers and Hunters it is H for H heparent sulfate and dermatin sulfate accumulate in hurlers and Hunters Hunter requires Clear Eye for hunting there is no cornea clouding in Hunters that is an important feature what is the inheritance of hunters what is the inheritance of hunters Hunters Hunter goes for hunting taking and acts acts is x-ling the recessive the rest of them are autosomal recessive okay the rest of them are autosomal recessive please do not confuse this Hunters with Huntington's Huntington is autosomal dominant okay Huntington is autosomal dominant Hunters is excellent recessive next one uh lysosomal storage disorders PDF you would get on the telegram group on my telegram Group after the class is over okay lysosomal storage disorders quickly having a look at those so in lysosomal storage we have sphingolipidosis we have the mucopolysaccharidosis and we have the pumpies right we said pumpies is the only glycogen storage disorder which is a lysosomal storage disorder as well it is acid maltase Alpha glucosidase which is deficient sphengo lipidosis gouchers okay coming to gouchers now remember the enzymes glucosidase and galactosidase how do you remember glucosidase and galactosidase Alpha and beta Alpha glucosidase Beta glucosidase Alpha galactosidase Beta galactosidase ticker so Alpha glucosidase we have already seen is acid Malta is Vala which is pumpies beta glucosidase has u in it gouchers has u in it so remember gouchers is beta glucosidase not galactosidase Alpha galactosidis and beta galactosidase Alpha is fabries and beta is crabs Alpha is fabries and beta is crab so when you have double b double b in crabs that tells you that it is beta fabry is single B that is not beta or fabric options remember fabric is like try try hexocal ceramide is what accumulates in favoris is dry okay so gouchers is you it is glucose cerebrosidase not galactose cerebrosidase also called as beta glucosidase taste sacks when I write taste sex the X and the a this is hexos amine it is a fabries is Alpha galactoseidase crabs is beta galactosidase or galactose cerebral cities myelinase when I write swingo myelinase remember the nip the nip helps me to remember it is sphingomyelinase very very important now coming to the features of this lysosomal storage disorders the various sphingolipidosis uh just give me I wanted to add a blank slide to write down just give me a minute foreign so what do we have is in Gauchos very very important and frequently Asked remember gouchers the patient says ouch ouch ouch ouch because the patient is in a lot of pain bone pain is a bad pain there is bone pain because the gouchers goes into the bone marrow so that leads to pancytopenia there can be anemia there is pancytopenia okay and when the patient is crying with the bone pain you give the tissue paper to wipe the tears so remember it has crumpled or wrinkled tissue paper appearance that is gouchers it has that erlin mayor flask deformity of the femur is what we see next that we have is Nimble picks what do we have in nimin picks and taste sacks so NP and Tay Sachs two word Wale single lipidosis they have two word of Allah defect that is the cherry red spot lemon pick is big big pick is big big so there is Big organs hepatosphenomegali present tesaxis tea tiny organs there is no hepatospino megali so cherry red with hepatocino megali NP cherry red without hepatocino megaly it is stay sax and in taste sacks we have a very important feature that is exaggerated startle reflects in a child because of hyperacusis okay because of hyperacusis we have the exaggerated startle reflex in taste sacks also remember in taste sack sack of onion onion it has onion peel appearance of of the lysosomes on electron microscopy and taste sacks is what gangliocidosis is is it gm1 is it gm2 is it gm3 what gangliocidosis is Tay sex remember t42 it is gm2 gangliocidosis again a previously asked question right and the next one that we have in name and picks what do we see in the nimin picks what are the findings what are the bodies that we see in nimin picks what are the findings what do we see in the name and picks so remember is like nimin picks is like no man okay so name and picks is like no man while crossing the zebra Crossing no man while crossing the zebra Crossing should pick the nose with finger okay that is Nim and pick with finger that is finger that is finger myelinase because then there can be foam coming out from the nose right the form coming out so we have the foam cells and we have the zebra bodies in zebra bodies and foam cells in lemon pics okay that is Neman pics so Gauchos next is fabries so remember fabry is like fabric okay fabry is like fabric what color fabric it is the Violet the pink color fabric is what we have it has those multiple Dots here what do the dots represent the skin lesions angio keratomas okay angio keratomas favorite is like Fabrics so remember it is x-linked recessive the sphingolipidosis which is x-link recessive is fabreeze so imagine you are going by a road and a shopkeeper comes behind you telling ma'am please take this cloth this is very very good cloth if you wear this cloth it will decrease your sweating so there is decreased sweating is a feature of fabries there is hypohidrosis decreased the sweating okay and there is early again there is increased risk of all the organ involvement there is stroke there can be Mi it can affect the kidney it can lead to nephrotic syndrome nephrotic syndrome and that is why what do you see in the URI Maltese cross appearance remember favorites has multi scrum appearance in the urine previous in ICT question okay previous inicity question so that is the story of Febreze okay that is the story of favorites so majority are done the next that is remaining is crabs what do we have in crabs remember that crabs are extinct from the earth that means in crabs the myelin is extinct there is total demyelination happening extinct to myelin is what is happening so even the optic nerve myelin gets affected so there is optic atrophy there can be optic nerve thickening because of the deposits plus bilateral thalamic hyper densities is what we see radiologically right so crap is extinct from the globe that means it is globoid bodies is what we see in crabs so remember a crab is extinct from the globe globoid body is extinct myelin optic atrophy and bilateral thalamic hyperdensities is what we see with crabs okay so everybody is clear with the sphingolipidosis the keywords that will help you to identify recessive G6PD deficiency is x-linked recessive blush neon is excelling recessive okay very very important uh next one I think we'll do this uh last one the table here for hormone sensitive versus lipoprotein or lipase okay so what do we have here a hormone sensitive lipase lipoprotein lipase which is catabolic which is anabolic out of these which is catabolic or which is anabolic out of these lipoprotein lipase lipoprotein and lipase is anabolic and hormone sensitive lipase is catabolic so if I say that lipoprotein a lipase is anabolic right then it is it is stimulated by which which hormone insulin will activate or insulin will block lipoprotein lipase right so this will insulin is an anabolic hormone insulin increases so anabolic it will increase the stores in the body so this will be stimulated by insulin okay this will be stimulated by insulin lipoprotein lipase so it is anabolic and remember it is stimulated by insulin hormone sensitive lipase basically causes the breakdown of the adipose in the body lipolysis so it will be stimulated by catechol amines by glucagon so look at this one hormone sensitive lipase present in the adipose site and it hydrolyzes the triacylglycerol in the adipose so basically this is catabolic so this will be active in the fasting State when we are not eating from outside that is glucagon lipoprotein and lipase is present in the capillaries it hydrolyzes the triacylycerol in the lipoproteins which lipoproteins which contain maximum triacylglycerol that is chylomicrons and bldl and it is active in the FED state that is it is activated by insulin so very very important remember that insulin activates the lipoprotein lipase so therefore in diabetic patients who have decreased insulin right so there would be decreased activity of lipoprotein lipase so therefore the vldl levels increase in diabetes because the lipoprotein lipase is not activated so the vldl is not broken down so remember the vldl levels increase ticket uh is this clear with everyone right hypertriglyceridemia is what we see because vldl have triglycerides maximum triglycerides okay so uh we will take a pause here for today and we will continue with the rest of the biochemistry tricks and the revision tomorrow I hope everybody each one of you were able to recollect it went a bit fast because we don't have much of time to go like you know Aram say and go into all the details right I'll share the PDF on the telegram group Dr Nikita's rat synapse and I would uh I would uh see you again tomorrow guys a lot of sessions Radiology plus course by cues fail at 10 am it's going to be the Radiology marathon and then we have the 6 PM kbmd basically to help you with the PSM numericals uh this is a free class and then 9 30 PM we will have the rest of the biochemistry class okay thank you so much everyone good night take care and keep studying keep revising and keep winning thank you

hello everyone happy evening a very good evening I hope and I believe all of you are doing well a quick note whether the audio visual is all good let me just check the live chat all right I believe or you can uh see me and hear me as well so here we are in the biochemistry Mega revision uh where we would try to revise uh all the important points and the must know points in Biochemistry and this is the first part itself this is the part one itself that we are doing here today right okay and apart from that we have a few other apart from that we have other free live classes also going on so on the unacademy app at 6 PM starting today we have planned for three classes uh so 6 pm today we had the class on physiology graphs and few numericals uh tomorrow 6 PM we will have a class on PSM numericals basically telling you the tricks on how to solve the questions fast and the mnemonics to remember the various the p-value sensitivity specificity all of that and on 26 it would be basically pre and para clinical subjects the clinical way there would be kbmd of that uh so clinical questions of first and second year uh second year subjects is what we are going to do I&#39;ve shared the links on the telegram group already we will have part two of the biochemistry Mega revision tomorrow at 9 30 PM okay uh yes thank you so much all these classes are planned because based on the feedback the inputs that I&#39;ve received from majority of you so thank you K and everyone else here a quick update on what&#39;s happening on the platform so the unlock is back uh we you have now uh 20 percent of available on medical PG subscriptions so fmg students can take for the fmg Mastery course we have for the next students as well and you have the code Dr Nikita for the 20 off you also have now the short shot 150 available at a very very reasonable price of 1600 with the credits that you have on the app you can get it for as low as 1200 so this is still uh 28 February we have this ongoing offer in the Aspire next 2023 batch uh basically it&#39;s a nine months course we are starting on February 28th we have a really good Educators to cater to your subjects it&#39;s gonna cover the theory the mcqs the clinical mcqs and everything for the current exam going students we have the final booster batch a one month rapid revision course now available at 4K so pre by queues most expected questions VTR is available in this final booster batch and for the fmd students we have started the batch on February 10th by April we will be finishing the theory and then in the rest of the two months we would be focusing on the McQ practice right so let us start with the first one so I&#39;ll be first starting with the topic of amino acids because this is a very very important and first we&#39;ll do the structure part of it then the amino acid disorders expect one question from that right so amino acid special groups so I&#39;ll be quickly telling you everything because there are so many things to be revised so we&#39;ll go a bit fast you can always come back to this video later and you can revise as well later on as well okay so remember that guanidinium group guanidinium G4 G is present in arginine Benzene group is present in phenylalanine when I say phenyl we know that it has that Benzene ring and tyrosine has a phenol group okay five tyrosine has a phenol group histidine is imidazole so remember it is high high means histidine has imidazole group Proline is pyrolidine Ryman Proline and pyrolidine methion and thiothio is The thio Ether linkage and tryptophan has a indole group so remember this tryptophan is like try the tri-color flag of India so India is indole and this is tryptophan so remember tryptophan has indole group and also remember that tryptophan try for vitamin B3 it forms vitamin B3 60 milligram of cryptofan forms 1 milligram of acid right so that is why whenever there&#39;s going to be a tryptophan deficiency there&#39;s going to be niacin deficiency like you have in conditions like hot laps carcinoid where all the tryptophan is going towards serotonin okay next one uh so which amino acids are most acidic and which are the most basic so thinking logically acidic acidic aspartic acid glutamic acid or acidic aspartic acid a a is very very acidic okay and the next that we have is a basic basic basically means Alkali you can remember it as Alkali Alkali is h a l so we have histidine Arginine and lysine which are the most basic arginine is the most basic so remember that a a are the ones which are the most acidic and most basic so if I tell you which is the most acidic aspartic acid which is the most basic again a for arginine okay a for arginine so remember Alkali is alkaline that is histidine Arginine and lysine Are alkaline that is basic now which are the essential amino acids and which are the non-essential amino acids for the essential amino acids we have um private Tim Hall okay few queries here what is the duration of this particular class this is going to be around 50 minutes one hour class part one next will be part two tomorrow is this enough for the last minute revision I would not say this is enough but this is the core this is like must know you cannot go to the exam Hall without knowing this I hope this answers all the queries that I&#39;ve been getting here in the live chat okay so private Tim Hall is the essential amino acids P for phenylalanine valine and remember T is three on in okay T is three on it it is not tyrosine uh I&#39;m really sorry could not share the PDF before the class should have done it but I forgot I&#39;ll do it after the class is over okay then you have t for tryptophan isoleucine methional histidine Arginine and Lucin and lysine some books also put h and a it is into a semi-essential so you can remember ah it comes as a surprise these are not essential these are like semi-essential so Arginine and histidine are some books uh put it into uh semi-essential now the next one that we have is so essential and semi-essential we know now now tell me that which are the amino acids which are both uh glucogenic and ketogenic amino acids which are both glucogenic and ketogenic are remember this is a tip to remember that which are both glucogenic and ketogenic it is tip what does tip stand for tryptophan isoleucin phenylalanide and the other t for tyrosine so basically all the aromatic amino acids phenylalanine tryptophan tyrosine and isolucine you can remember that I saw Lucin ISO Matlab acting or present in isoform that is two forms that is glucogenic and ketogenic so remember aromatic amino acids plus isoleucine these are both glucogenic and ketogenic also remember that which are the amino acids which absorb of the ultraviolet light those are the aromatic amino acids phenylalanine tyrosine tryptophan maximum is with tryptophan okay maximum is with tryptophan this is a previously asked question that if you replace ln9 with which amino acid then there will be light absorption at 280 nanometers that is tryptophan next one which are the pure ketogenic amino acids remember L and L okay L and L then what do we have is ketogenic so remember ketogenic is leucine and lysine and all the rest of them they go into glycogenic so remember which is ketogenic remember which is both if not any of that then that amino acid is glucogenic then that amino acid is glucogenic okay now going to which are the branched chain amino acids which are the branch chain amino acids remember a tree which is branching right a tree which is branching which is that tree this is a maple tree which has the branches right so why am I telling you maple tree because the defect in the branch in amino acid metabolism leads to maple syrup urine disease when do we Branch out into the multiple Generations right we Branch out into multiple Generations when we are live when we live when we are live so remember l i v what is l i v l i v is Lucin isolucin and valine remember L is not for lysine okay L is not for lysine so when I say isolution it is not lysine it is not lysine right out of that we saw that isolution is both is only ketogenic and valine is glucogenic right so you can also be asked a question like which of these is a branch chain amino acid and is both leukogenic and ketogenic you have the options remember it is isoleucine okay it is isoleucine so maple syrup urine disease is a defect in Branch chain ketoacid dehydrogenase one two three four letters one two three four letters voila enzyme msud is bckd and remember that What vitamin supplementation can help in msud it is vitamin B1 thymine is a very important part of bckd this bckd requires what other vitamins one plus two three two plus three five one plus two three two plus three five vitamin B1 B2 B3 B5 and it requires lipoic acid okay so vitamin B1 supplementation is what we give uh next we have is enzymes what are the various enzymes defective in the various amino acid disorders so what do we have phenyl Ketone urea as the term says it is phenylalanine hydroxylase phenylalanine hydroxylase deficiency tyrosinemia type 1 which is tyrosynosis remember one is first first is fumeral acetoacetate hydrolase that is f a a h maple syrup maple tree it is branching Branch chain Alpha keto acid dehydrogenase Al caponeuria is homogenicid epoxy days okay homogenicide oxidase alcaptainuria is a defect in which amino acid metabolism while Captain urea is a defect in tyrosine metabolism okay it&#39;s a defectant tyrosine metabolism albinism again is tyrosinase because milanin comes from tyrosine homocysteine urea type 1 that&#39;s a classical one so remember that homocysteine is Sister thionine synthesis which is What vitamin dependent homocysteine is like homo 16 so remember it is vitamin B6 this is dependent on vitamin B6 uh homocysteine urea type 2 is the other one that is B12 deficiency then you have tyrosinemia type 2 which is Rich norat syndrome two has two words richner hanat and remember 240 and the enzyme is that that is tyrosine amino transferase or tyrosine trans aminase tyrosine Amino transferase okay and when in which amino acid metabolism defect do we see hyperoxylurea remember glyoxylate that is basically glycine glycine metabolism defect May hyperoxyl urea okay remember that now what are the tyrosinemia types you have type 1 type 2 type 3 and you have your Hawkins urea type 1 is tyrosynosis this is the one which is called as hepato renal this is the one which increases the risk of HCC and F for first f a a h is the enzyme tyrosinemia type 2 that is richness syndrome is that this is what we already saw this has oculocutaneous involvement and which is the neonatal tyrosinemia that is tyrosinemia Type 3 which can be seen as a benign condition in the newborn because of the functional uh you know the function is less developed in the new bonds for the enzyme for hppd hydroxyphenyl pyruvate dioxygenase so remember in type three three four it is 4 hppd which is defective if it is a mutation in the gene for hppd remember h4h then it leads to Hawkins urea okay then it leads to Hawkins urea so remember one is first faah2 is that 3 is 4 4 hppd one is the one uh which is the most severe and it has what order it has cabbage odor it increases the risk of HCC remember cab car tire that means cabbage odor is seen in tyrosinemia and from the tire fumes are coming out that means tyrosinemia forms fumerate as the product you have fumaril acetoacetate hydros uh hydrolase next one looking at the urine order urine order my maple syrup means Maple syrupoga remember syrup is the sugar that means it has burnt sugar order okay it has burnt sugar order which is also called as caramelized sugar it means the same thing syrup contains sugar so maple syrup is burnt sugar Hawkins remember Hawk is a bird who is swimming in the swimming pool so Hawkins is swimming pool uh then you have sweaty feet sweaty feet is isovaloric when I write ISO valeric remember the SV is the sweat sweaty feet cabbage is tyrosinemia fumes multiple carboxylase deficiency this is the c a m c a m c a this is Tom cat urine so remember tom cat urine is multiple carboxylase deficiency phenyl phenyl is like f f is for M M is for f phenylketonuria is mousy order methylamineuria is fishy odor this is a previously asked question in one of the recent fmge exams okay so remember methylamine urea is uh is a rotting fish uh these are the ones that we have already covered here the odor in the various Okay order in the various amino acid disorders next one homocysteine urea is something which is very very important and remember few points about homocysteine urea clinical features are this patient habitus is similar to marfons that means this is a tall patient with the long fingers and what else do we have the keywords are early age am I early age stroke because homocysteine urea is like a pro atherosclerotic Cloud condition okay it fastens the atherosclerosis like Marfan this also has the lens subluxation but where do you have the lens of luxation and homocysteinuria it is opposite of marfons where do you have in marfons remember marfons is like Mar fan is present up in the ceiling and we say everybody fan out so in Marfan it is up and out so in homosystem area it is down and in so it is down and in that means it is infero medial okay that means it is inferometer inferonasal is what you should remember right so and here the IQ also can be affected in homocysteine urea so few questions on homocysteineuria homocysteineuria is formed from which amino acid homocysteine urea this is basically a reversible reaction that is present here methion informs homocysteine homocysteine forms methionine so remember homocysteine is formed from methionine next Once the homocysteine is formed either it can go to form methionine back to methionine or it can go ahead and it can form cysteine right so remember it is m h c like your major histocompatibility complex H can form m h can form C that means it can form methional or it can form cysteine and of to form the cysteine this is vitamin B6 dependent so homocysteine is basically homo 16 getting converted to cysteine that is again like 16. this requires vitamin B6 okay requires vitamin B6 so sister thionine synthesis is vitamin B6 right so when there is homocysteine urea this step is blocked and that is why homocysteine gets elevated so therefore if you get a question that in homocysteine urea uh what what should be supplemented and what should be stopped remember that is supplementation needs to be given methionine should be restricted because methionine forms homocysteine we do not want more homocysteine so restrict methionine gives cysteine in homocysteine urea okay and for the conversion of homocysteine to mithyan so this requires methyl Cobalt a mind that means vitamin B12 and vitamin B12 bhakti the post test is at 10 30 I believe right it&#39;s not 10 I&#39;m sorry okay so what do you need to remember is vitamin B12 is required for conversion of homocysteine to methion and methyl Cobalt amide and b12 and B9 that is B12 and folic acid are very interrelated right so remember this is B9 and b12 So What vitamin deficiencies basically can lead to homocysteine urea remember the multiples of three but not starting with three that is 6 9 12 vitamin B6 vitamin B9 vitamin B12 but not vitamin B3 niacin deficiency will not cause homocysteine urea okay 6 9 12 6 is the classical one okay six is the classical one if you have to choose one so homocysteine urea intellectual disability stroke Mi marfelloid habitus lends the luxation which is infero medial kyphosis skeletal formation deformities and osteoporosis decrease methion increase the cysteine you have B6 B9 and b12 supplementation is what we need okay and this is what is given here basically TK next one let&#39;s go to phenylalanine metabolism now phenyl Allen 9 it forms tyrosine by phenylalanine hydroxylase so that is why when this enzyme is deficient this leads to phenyl Ketone urea so there would be phenylbutyrate of phenyl lactate that would be formed and so there will be phenylketone which will increase which gives the mousy odor or the musty odor okay it gives the mousy or the musty odor what are the clinical features in phenyl ketoneuria remember that the clinical features are blonde hair is a feature given with a mental retardation IQ is decreased okay another question if the mother has phenyl Ketone urea during pregnancy that means what is the teratogenic effect on the child it can lead to microcephaly okay it can lead to microcephaly next one that we have what is the other thing that this phenylalanine hydroxylase requires it is a tetrahydrobiopterin that is bh4 tetrahydro B for biopterin is required okay so remember that can lead to non-classical phenyl ketoneuria so tyrosine tyrosine so what amino acid becomes essential in a phenyl ketoneuria it is tyrosine normally tyrosine can be formed in the body from phenylalanine but if there is a deficiency at this step tyrosine will not be formed so tyrosine will become essential next one that we have is uh tyrosine goes ahead and forms dopa dopa forms dopamine norepinephrine epinephrine so basically the catechol amines all of these are formed from tyrosine you can remember this as tyrosine sounds similar to thyroxine so when thyroxine is increased in the body hyperthyroidism we know that there is a lot of catecholamine reaction sensitivity there so remember that thyroxyl and catecholamines go together and both of them are formed from tyrosine so a tyrosine by tyrosine hydroxylase forms dopa even the styrosine hydroxylase requires tetrahydrobiopterin so all the three aromatic amino acids hydroxylase phenylalanine hydroxylase tyrosine hydroxylase tryptophan hydroxylase and you have nitric oxide synthase these require tetrahydrobiopter and what is the reaction which basically converts a norepinephrine to epinephrine it is a methyl transferase that is methylation so remember that norepinephrine has no methyl group when you add a methyl group then it becomes epinephrine which is a methyl donor in the body it is it is s adenosyl methionine that is Sam you can see that methion and here is forming homocysteine as adenosine homocysteine so methionine is forming homocysteine okay dopamine forms norepinephrine by hydroxylase dopamine hydroxylase What vitamin is required for hydroxylases hydroxy is water where is the water present water is present in the sea so remember this requires vitamin C hydroxylation dopa forms dopamine by dopa B carboxylase so always remember this point that whenever an amino acid is forming an amine amino acid forming an amine that means you are removing the acid group so it is the carboxylation reaction this requires vitamin B6 okay this requires vitamin B6 the carboxylase requires vitamin B6 okay uh so now going to the next one so phenylalan9 uh tyrosine this can lead to phenylketon urea if this is stopped tyrosine forms dopa from dopa you have Milan and also by the action of tyrosinase so albinism is tyrosine deficiency remember albinism melanin is formed from tyrosine so it is albinism from tyrosinees dopa to dopamine we said it is vitamin B6 dopamine to norepinephrine vitamin C hydroxylase this is methylation this is Sam even homogenic acid comes from tyrosine that is why Al captonuria homogeneity oxidase deficiency is tyrosine deficiency key word for Al Capone urea remember Alka just rearrange that it is kala so you get kala urine on standing black urine on standing with El capto Nuria with pigmentation of the cartilage with okronosis and the patient is at risk of developing arthropathies early age arthropathies with Al captonuria remember it is autosomal recessive majority of these amino acid disorders are autosomal recessive okay they are autosomal recessive right going on to the next one this we have already seen phenylketonuria so decrease phenylalani because it is not getting metabolized so no need of giving that increase the tyrosine in the diet give bh4 supplementation Fair complexion mousy odor intellectual disability is what we see the phenylketones will increase in the urine okay next one uh next one this is what you are seeing here the same thing has been shown here remember that tyrosine forms Fumarate and acetoacetate types we have already seen going to amino acid products amino acid products quickly revising this is the same thing which is given here this we have already seen Millennial and thyroxine is also from tyrosine remember that what does tryptophan form trypto is tried to Tri is vitamin B3 and trypto is to serotonin and melatonin okay remember not milanin melanin is from tyrosine right absolutely right tire is black in color so remember black is melanin serotonin melatonin comes from cryptophan STD forms carnosine histamine is from histidine by which reaction you are forming the amine so this is decarboxylation okay glycine is required for a lot of things most important remember it&#39;s required for hemosynthesis and many other things is where we would see that and glutamate is G4 Gaba glutathione okay glutathione also requires glutamate very very important you&#39;ll see this question very frequently in the exam nitric oxide synthesis requires Arginine that is nitric oxide okay nitric oxide arginine is required for urea also and creatine also okay urea and creatine also so remember that if you get a question that arginine is required for synthesis of creatine urea nitric oxide all of the above remember it is all of the above okay do not be in a hurry to Mark the answer key Arginine in a nitric oxide always take the last option if you have all of the above okay going on to the next one so a quick revision of the products melanin is from tyrosine melatonin is tryptophan t3t4 thyroxine is again tyrosine histamine is from histidine Gaba is from glutamide B3 tryptophan creatine requires what all amino acids create always try to create a magnificent results mag are the amino acids required so you have methionine Arginine and glycine what are the three amino acids methionine Arginine and glycine are required then serotonin is cryptophan tytical amines thyroxine Vala that means it comes from tyrosine okay it comes from tyrosine the next one creatine synthesis what are the steps in creatine synthesis where does it occur first step is in the muscle and the liver or in the kidney first step is in the muscle kidney or liver where is the first step for creatine synthesis it&#39;s in the liver everybody&#39;s saying liver the steps of creatinine synthesis the first step is in the kidney then in the liver and then in the muscle easy to remember alphabetically remember h i j k l m so kidney liver muscle so first it starts with the kidney the first thing to form in the kidney is what is getting formed is guanidino acetate guanidino acetate guanidinium containing amino acid was Arginine so this requires Arginine and glycine okay so remember it is KLM is what you need to remember so guys look at the amino acids required for creatine you have Arginine Glycine and methionine that is mag result okay that is mag result uh next one what do we have is a glutathione glutathione what does glutathione require what does the glutathione require which amino acids remember glutathione is three that is g c g glutamate glutamic acid is required thiocontaining cysteine is a sulf hydro group containing thiocontaining and again you have glycine here okay again you have glycine here so look at this one uh this is glycine assistine and glutamate that is g c g that is for glutathione and which is the most functional the most active amino acid here for reduced glutathione the one which contains the sulfhydryl group reduce the glutathione is the cysteine cystinavala is the most active Okay cysteine is the most functional site there purine and pyrimidines what do they require remember that purines are two rings they have two rings pyramiding have a single ring okay pyramidine have a single ring so purines are two rings so they require more amino acids as compared to pyrimidines so Puri imagine like which are the purines those are the A and the g adenine and guanine these are the purines right and then you have when you have egg puree someone who has not had egg anytime has an egg puree gets a gag reflex so remember gag are the amino acids required you have glutamine Glycine and aspartate that is g a g okay that is g a g is required for purine in pyramidi we do not require pyrimidine so one amino acid is removed from gag the G is removed that is the glycine is not required you have glutamine and aspartate remember it is glutamine and aspartate not aspar Gene and glutamate it is glutamine and asparted which is required for purine and pyrimidine next one coming to a glycogen storage disorders are very very important so we have seen about the amino acid disorders very very important phenylketonuria El captonuria albinism then you have maple syrup urine disease a question on this uh generally comes in the exam very frequently next one glycogen storage disorders glycogen storage disorders quick points type 1 is one gurkis okay type one is one agar cheese and G 4G gurkis is glucose six phosphatase is the enzyme deficient so one gurkis is glucosa six phosphatase can you tell me this is a previously asked question glucose 6-phosphatase is a marker of which cell organelle glucose 6-phosphatase is a marker of which cell organelle it is a marker of endoplasmic reticulum okay it&#39;s a marker of endoplasmic reticulum very good so in what the key is the glucose 6-phosphatase is gone and this glucose 6-phosphatase what is it required for is it required for glycogenolysis is it required for gluconeogenesis or this is required for both remember this is required for both okay it&#39;s an enzyme required for both so when glucose 6-phosphatase is not there that is one gurkis both the glucose production pathway will be affected in the body and that is why there will be severe fasting hypoglycemia which will not be responsive even to glucagon challenge test will not be able to increase the glucose levels Plus everywhere the glycogen is deposited so there is hepatomegaly that is renomegaly there is hyperlipidemia so fat deposited in the cheeks imagine like chubby chicks it is called as doll like faces dog like fishies is another important feature as we have lactic acidosis and hyperuricemia okay these are very very important features of one gurkis okay yes that was the trick that we had posted on Instagram today itself in the 50 day challenge Victorious people can always make history today okay so one garkis pumpes curries Andersons mercado&#39;s hers and taruis these are the glycogen storage disorders one by one Victorious people can always make history today okay they can always make history today also okay then you have type 1B where is there is the transporter defect only okay the transporter Defector only this will have recurrent infections also type 2 is uh pumpies okay type 2 is pumpies Victorious People pumpies affects the pump in the body that is the heart so that is why there will be cardiomegali and heart failure heart requires fatty acid right so remember that the enzyme which is defective here is acid maltase acid maltase which is also your lysosomal enzyme so pompase is the only one which is a pump is the only one which is the glycogen storage disorder which is a lysosomal storage Disorder so it is actually Alpha glucosidase okay this is Alpha glucosidase enzyme okay next one type 3 the third alphabet is C this is Corey&#39;s also called as Forbes or limit dextrinosis has a defect of a d branching enzyme okay remember here also there is muscle weakness uh there is hepatomegaly due to accumulation of branched chain polysaccharide because the D branching is not happening so you will see the short branches which will be present that will lead to hepatomegaly Type 4 remember when I write 4 and I make it like a 4 is like a so that is Type 4 is under sensor disease remember Anderson dies at the sun&#39;s age that means that is at five years young age because of there is liver cirrhosis remember there is cirrhosis in Andersons okay and here there is a problem with branching Anderson branching Corey&#39;s D branching so what happens in Anderson&#39;s you would see long polysaccharide chain without branches that will accumulate so this will precipitate in the liver and that will cause cirrhosis so remember a for a this is also called as a myelopectinosis because it is like amylopectin the long chain type of 5 is the MAC address is the muscle glycogen phosphorylase hers is the hepatic glycogen phosphorylase mccardless muscle hers hepatic okay these are type 5 and type 6. muscle is gone so exercise induced cramps muscle breakdown okay Rhabdomyolysis myoglobin urea the renal failure yoga and very important remember there is a second wind phenomenon okay Second Wind phenomenal that means after this you know a break in the exercise the person can exercise again it&#39;s because of the increased cardiac output The increased blood flow to the muscles that&#39;s a compensatory mechanism so remember second when the phenomenon is seen in mcardle&#39;s disease WKA Ulta is m when you have uh the liver hepatic it&#39;s a mild hypoglycemia versus one gurkis which has severe hypoglycemia type 7 is taruis remember when I write T it has seven in it so remember taru is a seven and taruis is like tone rbcs so there is hemolytic anemia in taruese whenever the rbcs are affected uh the cycle that we are thinking about is glycolysis so glycolysis enzyme it is phosphofructokinase so this affects the muscle phosphofructokinase so exercise Wale cramps or hemolytic anemia is what we would see here so that is phosphofructokinase affected so these are the glycogen storage disorders have a look at this image also given in your first aid so what happens here is uh look at the first one what do you have at the first one the second is glycogen synthase this is how the glycogen is formed then occurs the branching okay then occurs the branching basically right and then what happens this one which you see where the glycogen can be uh degraded breakdown in the lysosome this is going to be pumpies okay this is going to be pompies then the next that we have which is glycogen phosphorylance defective it is a MAC address and where you have the short branches which are not able to be removed this is chorized the D branching is affected okay the D branching is affected right hurlers and Hunters remember these come under mucopolysaccharidosis hurlers Hunters her loss is l i duronides Hunters is ideuronate sulfur taste TNT L and L and in both of them it is heparent sulfate and dermatin sulfate which accumulate so in hurlers and Hunters it is H for H heparent sulfate and dermatin sulfate accumulate in hurlers and Hunters Hunter requires Clear Eye for hunting there is no cornea clouding in Hunters that is an important feature what is the inheritance of hunters what is the inheritance of hunters Hunters Hunter goes for hunting taking and acts acts is x-ling the recessive the rest of them are autosomal recessive okay the rest of them are autosomal recessive please do not confuse this Hunters with Huntington&#39;s Huntington is autosomal dominant okay Huntington is autosomal dominant Hunters is excellent recessive next one uh lysosomal storage disorders PDF you would get on the telegram group on my telegram Group after the class is over okay lysosomal storage disorders quickly having a look at those so in lysosomal storage we have sphingolipidosis we have the mucopolysaccharidosis and we have the pumpies right we said pumpies is the only glycogen storage disorder which is a lysosomal storage disorder as well it is acid maltase Alpha glucosidase which is deficient sphengo lipidosis gouchers okay coming to gouchers now remember the enzymes glucosidase and galactosidase how do you remember glucosidase and galactosidase Alpha and beta Alpha glucosidase Beta glucosidase Alpha galactosidase Beta galactosidase ticker so Alpha glucosidase we have already seen is acid Malta is Vala which is pumpies beta glucosidase has u in it gouchers has u in it so remember gouchers is beta glucosidase not galactosidase Alpha galactosidis and beta galactosidase Alpha is fabries and beta is crabs Alpha is fabries and beta is crab so when you have double b double b in crabs that tells you that it is beta fabry is single B that is not beta or fabric options remember fabric is like try try hexocal ceramide is what accumulates in favoris is dry okay so gouchers is you it is glucose cerebrosidase not galactose cerebrosidase also called as beta glucosidase taste sacks when I write taste sex the X and the a this is hexos amine it is a fabries is Alpha galactoseidase crabs is beta galactosidase or galactose cerebral cities myelinase when I write swingo myelinase remember the nip the nip helps me to remember it is sphingomyelinase very very important now coming to the features of this lysosomal storage disorders the various sphingolipidosis uh just give me I wanted to add a blank slide to write down just give me a minute foreign so what do we have is in Gauchos very very important and frequently Asked remember gouchers the patient says ouch ouch ouch ouch because the patient is in a lot of pain bone pain is a bad pain there is bone pain because the gouchers goes into the bone marrow so that leads to pancytopenia there can be anemia there is pancytopenia okay and when the patient is crying with the bone pain you give the tissue paper to wipe the tears so remember it has crumpled or wrinkled tissue paper appearance that is gouchers it has that erlin mayor flask deformity of the femur is what we see next that we have is Nimble picks what do we have in nimin picks and taste sacks so NP and Tay Sachs two word Wale single lipidosis they have two word of Allah defect that is the cherry red spot lemon pick is big big pick is big big so there is Big organs hepatosphenomegali present tesaxis tea tiny organs there is no hepatospino megali so cherry red with hepatocino megali NP cherry red without hepatocino megaly it is stay sax and in taste sacks we have a very important feature that is exaggerated startle reflects in a child because of hyperacusis okay because of hyperacusis we have the exaggerated startle reflex in taste sacks also remember in taste sack sack of onion onion it has onion peel appearance of of the lysosomes on electron microscopy and taste sacks is what gangliocidosis is is it gm1 is it gm2 is it gm3 what gangliocidosis is Tay sex remember t42 it is gm2 gangliocidosis again a previously asked question right and the next one that we have in name and picks what do we see in the nimin picks what are the findings what are the bodies that we see in nimin picks what are the findings what do we see in the name and picks so remember is like nimin picks is like no man okay so name and picks is like no man while crossing the zebra Crossing no man while crossing the zebra Crossing should pick the nose with finger okay that is Nim and pick with finger that is finger that is finger myelinase because then there can be foam coming out from the nose right the form coming out so we have the foam cells and we have the zebra bodies in zebra bodies and foam cells in lemon pics okay that is Neman pics so Gauchos next is fabries so remember fabry is like fabric okay fabry is like fabric what color fabric it is the Violet the pink color fabric is what we have it has those multiple Dots here what do the dots represent the skin lesions angio keratomas okay angio keratomas favorite is like Fabrics so remember it is x-linked recessive the sphingolipidosis which is x-link recessive is fabreeze so imagine you are going by a road and a shopkeeper comes behind you telling ma&#39;am please take this cloth this is very very good cloth if you wear this cloth it will decrease your sweating so there is decreased sweating is a feature of fabries there is hypohidrosis decreased the sweating okay and there is early again there is increased risk of all the organ involvement there is stroke there can be Mi it can affect the kidney it can lead to nephrotic syndrome nephrotic syndrome and that is why what do you see in the URI Maltese cross appearance remember favorites has multi scrum appearance in the urine previous in ICT question okay previous inicity question so that is the story of Febreze okay that is the story of favorites so majority are done the next that is remaining is crabs what do we have in crabs remember that crabs are extinct from the earth that means in crabs the myelin is extinct there is total demyelination happening extinct to myelin is what is happening so even the optic nerve myelin gets affected so there is optic atrophy there can be optic nerve thickening because of the deposits plus bilateral thalamic hyper densities is what we see radiologically right so crap is extinct from the globe that means it is globoid bodies is what we see in crabs so remember a crab is extinct from the globe globoid body is extinct myelin optic atrophy and bilateral thalamic hyperdensities is what we see with crabs okay so everybody is clear with the sphingolipidosis the keywords that will help you to identify recessive G6PD deficiency is x-linked recessive blush neon is excelling recessive okay very very important uh next one I think we&#39;ll do this uh last one the table here for hormone sensitive versus lipoprotein or lipase okay so what do we have here a hormone sensitive lipase lipoprotein lipase which is catabolic which is anabolic out of these which is catabolic or which is anabolic out of these lipoprotein lipase lipoprotein and lipase is anabolic and hormone sensitive lipase is catabolic so if I say that lipoprotein a lipase is anabolic right then it is it is stimulated by which which hormone insulin will activate or insulin will block lipoprotein lipase right so this will insulin is an anabolic hormone insulin increases so anabolic it will increase the stores in the body so this will be stimulated by insulin okay this will be stimulated by insulin lipoprotein lipase so it is anabolic and remember it is stimulated by insulin hormone sensitive lipase basically causes the breakdown of the adipose in the body lipolysis so it will be stimulated by catechol amines by glucagon so look at this one hormone sensitive lipase present in the adipose site and it hydrolyzes the triacylglycerol in the adipose so basically this is catabolic so this will be active in the fasting State when we are not eating from outside that is glucagon lipoprotein and lipase is present in the capillaries it hydrolyzes the triacylycerol in the lipoproteins which lipoproteins which contain maximum triacylglycerol that is chylomicrons and bldl and it is active in the FED state that is it is activated by insulin so very very important remember that insulin activates the lipoprotein lipase so therefore in diabetic patients who have decreased insulin right so there would be decreased activity of lipoprotein lipase so therefore the vldl levels increase in diabetes because the lipoprotein lipase is not activated so the vldl is not broken down so remember the vldl levels increase ticket uh is this clear with everyone right hypertriglyceridemia is what we see because vldl have triglycerides maximum triglycerides okay so uh we will take a pause here for today and we will continue with the rest of the biochemistry tricks and the revision tomorrow I hope everybody each one of you were able to recollect it went a bit fast because we don&#39;t have much of time to go like you know Aram say and go into all the details right I&#39;ll share the PDF on the telegram group Dr Nikita&#39;s rat synapse and I would uh I would uh see you again tomorrow guys a lot of sessions Radiology plus course by cues fail at 10 am it&#39;s going to be the Radiology marathon and then we have the 6 PM kbmd basically to help you with the PSM numericals uh this is a free class and then 9 30 PM we will have the rest of the biochemistry class okay thank you so much everyone good night take care and keep studying keep revising and keep winning thank you

Transcript for:Biochemistry Mega Revision - Part 1

Transcript for:
Biochemistry Mega Revision - Part 1