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Biochemistry-mixed

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medmaestro's version from 2016-01-18 18:00

Section

Biochemistry
PORPHYRIAS

Hepatic Porphyrias

Question Answer
Acute Intermittent Porphyria- photosensitivity absentUroporphyrinogen – I – Synathase
Variegate PorphyriaProto Porphyrinogen Oxidase
Hereditary CoproporphyriaCopro Porphyrinogen Oxidase
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Erythropoietic Porphyria

Question Answer
Erythropoietic Proto PorphyriaFerrochelatase
Congenital Erythropoietic Porphyria-photosensitivity absentUroporphyrinogen III Synathase
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Combined

Question Answer
Porphyria Cutanea TardaUroporphyrinogen decarboxylase
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Classification of L-α Amino acids on the basis of side chains

Question Answer
With aliphatic side chainsglycine,alanine,valine,leucine,isoleucine
With side chains containing hydroxyl group (-OH)serine,threonine,tyrosine
With side chains containing sulphur atomscysteine,cystine,methionine
With side chains containing acidic groups or their amidesaspartic acid,asparagine,glutamic acid ,glutamine
With side chains containing basic groupsarginine,histidine,lysine
With side chains containing aromatic ringshistidine,phenylalanine,tyrosine,tryptophan
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Classification of amino acids based on structure

Aliphatic

Question Answer
Monoamino mono carboxylic acids -Simple AAglycine,alanine
Monoamino mono carboxylic acids -Branched chain AAvaline,leucine,isoleucine
Monoamino mono carboxylic acids -Hydroxyl AAserine,threonine
Monoamino mono carboxylic acids -Sulphur containing AAcysteine,methionine
Monoamino mono carboxylic acids -AA with amide groupasparagine,glutamine
Mono amino di carboxylic acidsaspartic acid,glutamic acid
Dibasic mono carboxylic acidslysine,arginine
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Question Answer
AROMATICphenylalanine,tyrosine
HETEROCYCLICtryptophan , histidine
IMINOproline,hydroxyl proline,selenocysteine
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Derived AA

Question Answer
FOUND IN PROTEINShydroxy proline,hydroxyl lysine
Non protein AAornithine,citrulline,homocysteine
Non alpha amino acidsGABA,Beta alanine
Ketogenic aino acidsleucine,lysine
Glucogenicvaline,cysteine,serine,alanine,histidine,threonine,arginine,glycine,glutamate,proline/hydroxyproline
Both glucogenic and ketogenicisoleucine,tyrosine,tryptophan,phenylalanine
Hypoglycemic AALeucine(ketogenic) & arginine (insulin releaser)
Glutamate is the precursor forarginine,proline,glutamine
Most human AA areL alpha Amino acids
Human D –Amino acidsD-aspartate in brain ;D-serine in fore brain
Molecules with 0 chargezwitter ions
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Classification of amino acids based on side chain

Hydrophobic

Question Answer
Hydrophobic tryptophan ,valine,methionine,leucine,isoleucine,alanine,phenylalanine,proline
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Hydrophilic

Question Answer
Uncharged polar side chainserine , asparagines,threonine,cysteine,tyrosine,glycine,glutamine
Acidic side chain (positive R chain )aspartic acid , glutamic acid (Tyrosine is mildly acidic )
Basic side chain (negative R chain )histidine ,arginine (most basic ),lysine
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Question Answer
Alpha amino acidsornithine,citrulline,arginosuccinic acid,T3,T4,S-Adenyl methionine,homocysteine,homoserine,3,4-dihydroxyphenylalanine,creatinine,ovothiol,azaserine
Non alpha amino acidsbeta alanine,beta amino butyric
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VITAMIN DEFICIENCY
Question Answer
B1(THIAMINE)CHRONIC PERIPHERAL NEURITIS,WERNICKE’S ENCEPHALOPATHY,KORSAKOFF’S PSYCHOSIS
B2(NIACIN)DEMENTIA
B6(PYRIDOXINE)EPILEPTIFORM CONVULSIONS IN INFANTS
PANTOTHENIC ACIDBURNING FOOT SYNDROME
VIT B12(CYANOCOBALAMINE) SUBACUTE COMBINED DEGENERATION (SACD)
FOLIC ACIDNEURAL TUBE DEFECTS
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TONGUE CHANGES IN VIT B DEFICIENCIES

Question Answer
Riboflavin defmagenta tongue
Niacin defbeefy red tongue/fiery red tongue
B12 defbaldness of tongue
Folic acid defpainful tongue
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Other vit related questions
Question Answer
Vit def in pancreatitisvit A
Milling of rice causes loss ofThiamine(vit B1),Vit B2-Riboflavin,Protein
Parboiling preservesVIT B1-thiamine in rice
Riboflavin def is associated withpyridoxine B6 def
Vit E def is associated withmild haemolytic anemia
Vit Emost potent antioxidant
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Allosteric inhibition
Enzymeallosteric inhibitorallosteric activator
Glutamate dehydrogenaseATP,NADHADP
HexokinaseATP,G-6-PADP
Pyruvate carboxylaseADPAcetyl Co A
PROTEIN KINASE----c AMP
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Competitive inhibition
ALL COMPETITIVE INHIBITORS ARE COMPLEX COMPOUNDS AND NOT SIMPLY METALLIC IONS.
Enzymecompetitive inhibitor
Lactate dehydrogenase oxamate
Aconitasetransaconitate
Succinate dehydrogenasemalonate
HMG-CoA ReductaseHMG
Dihydrofolate reductaseamethopterin
Xanthine oxidaseallopurinol
Folate reductasemethotrexate,trimethoprim,pyrimethamine
Adenylosuccinate synthetase6-Mercaptopurine
Thymidylate synthetase5-Fluorouracil
DNA Polymerasecytosine arabinoside
Neuraminidaseoseltamivir
Phosphoribosyl amido transferaseazaserine
Transpeptidasepenicillin
Pteroid synthetasesulphonamide
Mono amine oxidase (MAO)MAO inhibitors like Amphetamine
Acetyl choline esterasePhysostigmine
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Question Answer
Uncompetitive inhibitioninhibitor can bind only to ENZYME-SUBSTRATE COMPLEX and NOT to isolated enzymes. Vmax & Km- BOTH are decreased
Competitive inhibitionacts on active site,reversible inhibition,Km increased Vmax –no change
Non competitive inhibitionirreversible inhibition,may or may not act on active site,Km –no change,Vmax-decreased
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ACUTE PHASE REACTANTS
BLOOD COMPONENTSTORAGE TEMP
Whole blood,packed RBCs1-6 deg C
Platelets ,Leukocyte concentrate20-24 deg C
FFP,Cryoprecipitate<-30 deg C
AlbuminRoom temperature
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Blood can be stored in
Question Answer
Citrate-phosphate-dextrose(CPD)for 3 weeks
CPD-Adeninefor 5 weeks
Adsolfor 7 weeks
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Kidney least affected in acute GVHD.therefore not biopsied in acute GVHD.

ALL ENZYME DEFICIENCY DISEASES

 

DISEASEDEFICIENT ENZYME
Von Gierkes Disease IaGlucose 6 phosphatase
Von Gierkes Disease IbGlucose 6 phosphatase translocase
Pompes DiseaseAcid maltase / Lysosomal acid alpha glucosidase
Cori’s Disease/Forbes/Limit dextrinosisLiver and muscle debrancher enzyme
Andersons Disease/AmylopectinosisBranching enzyme
McArdles DiseaseMuscle phosphorylase
Her’s DiseaseHepatic phosphorylase
Tarui DiseasePhosphofructokinase
Fanconi/ Bickels DiseaseGlucose transporter II
AlbinismTyrosinase
Neiman – Picks DiseaseAcid sphingomyelinase
Metachromatic leukodystrophy Aryl sulphatase
Gauchers DiseaseAcid beta glucosidase
Orotic aciduriaOrotic phosphoribosyl transferase
GalactosemiaGalactose 1 phosphate uridyl transferase ; Galactokinase ; UDP galactose 4 epimerase
Fructose intoleranceFructose 1,6- biphosphatase
Essential fructosuriaFructokinase
Krabbe’s DiseaseBeta galactosidase
Fabry’s DiseaseAlpha galactosidase
Farbers Disease Acid ceramidase
Forbe’s Disease Debranching enzyme
MPS IAlpha L Iduronidase
MPS IIIduronate sulphatase
Tay sachs DiseaseBeta Hexosaminidase A
Sandhoff;’s DiseaseBeta Hexosaminidase A and B
Tyrosinemia IFumaryl acetoacetate hydrolase
Tyrosinemia IITransaminase
MSUDBranched chain alpha ketoacid decarboxylase
Homocystinutia ICystathionine synthetase
Homocystinuria IIN5 Methyl THF methyl transferase
SialidosisNeuraminidase
GM1 GangliosidosisBeta galactosidase
Essential pentosuriaNADP Dependant L Xylitol dehydrogenase
Refsum’s DiseasePhytanate alpha oxidase
CystinosisCysteine reductase
Lesch Nyhan Syndrome HGPRTase – Complete deficiency
Kelley – Seegmiller syndromeHGPRTase – Partial deficiency
Acute intermittent porphyriaUroporphyrinogen I synthetase
Congenital erythropoetic porphyriaUroporphyrinogen III synthetase
Porphyria cutanea tardaUroporphyrinogen decarboxylase
Variegate porphyriaProtoporphyrinogen oxidase
Erythropoetic protoporphyriaFerrochelatase
HyperammonemiaICarbamoyl phosphatase synthetase I
Hyperammonemia IITranscarbamoylase
CitrullemiaArginosuccinate synthetase
Arginosuccinic aciduriaArginosuccinase
ArginemiaArginase
FucosidosisAlpha fucosidase
Canavans DiseaseN Asperto Acylase
Adrenoleucodystrophy Acyl CoA synthetase
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