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125 Cards in this Set
- Front
- Back
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What are the two major mechanisms of amino acid breakdown?
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1) Transamination 2) Oxidative deamination
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AA biosynthesis of pyruvate + glutamate to a-ketoglutarate + aa via?
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aa aminotransferase
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What do ketogenic aa break down to?
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Acetyl CoA
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Activation of PDH phosphatase does what to PDH complex in turning pyruvate to Acetyl CoA?
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Activate
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Inability to make Hydroxymethylbilane Synthase?
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Acute Intermittent Porphyria
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Which Porphyria as high ALA and urine darkening, therefore CNS problems?
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Acute Intermittent Porphyria
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Salvage purine synthesis pathway for making AMP?
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Adenine + PRPP by Adenine PRT
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What are the main nitrogen transporters from muscle to liver?
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Alanine
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What are the main nitrogen transporters in blood?
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Alanine, Glutamine
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What are 2 drugs used for gout (hyperuricemia) - uric acid underexcretors?
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Probenecid, Sulfinpyrazone
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What proteolysis are on luminal surface for further aa cleavage?
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Aminopeptidase, Carboxypeptidase
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In transmination moves NH3+ from amino acid onto a-ketoglutarate via what enzyme?
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aminotransferase
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Process where Krebs cycle intermediates replenished by insertion within cycle
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Anaplerosis
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What block pyrimidine synthesis?
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Antineoplastic drugs
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Where does typsin cleave (after double bond)?
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Arg, Lys
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De Novo purine synthesis - what is added to turn IMP to AMP?
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Asp, GTP
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What inhibits PKF-1 ?
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ATP, Citrate
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What inhibits PDH complex, by ACTIVATING PDH kinase?
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ATP, NADH, Acetyl-CoA
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What vitamin does Cystathionine Synthase require?
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B6
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What can be used to treat disorders in urea cycle?
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Benzoate, Phenylbutyrate
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In Heme degradation what is formed to aid in excretion from body via bile?
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Bilirubin diglucuronide
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What is missing that causes infants to have jaundice?
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Bilirubin glucuronyl transferase
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In Heme degradation what is formed to aid in moving bilirubin to liver?
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Bilirubin-Albumin complex
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What activates PDH phosphatase?
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Ca2+
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What activates PDH complex or turning pyruvate to acetyl CoA?
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Ca2+, Pyruvate
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What is the rate limiting step in Urea Cycle?
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Carbamoyl phosphate synthesis
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What is enzyme of rate limiting step in pyrimidine synthesis?
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Carbamoyl phosphate synthetase II (neg. - UTP, pos. - ATP, PRPP)
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What are the 3 thermodynamically favorable reaction enzymes of the Kerb's Cycle (TCA)?
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Citrate Synthase, Isocitrate Dehydrogenase, a-ketoglutarate
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What moves across membrane seperating mito matrix and cytosol in Urea Cycle carrying NH4+ group?
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Citrulline
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What inhibits ETC at last stage?
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CN, CO
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What is the PDH complex composed of?
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Cofactors E1-E3
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In ETC what links complex 1,2 with 3?
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CoQ
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What uses lactate instead of alanine in transport of NH4+ from muscle to liver?
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Cori cycle
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What is the only enzyme exclusive to mito matrix side in urea cycle and stimulated by NAC?
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CPS (carbamoyl phosphate synthetase)
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What are the two final products of pyrimidine synthesis?
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CTP, dTMP
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What can be used to test for glucose in urine?
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Cu
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What is not functional in homocystinuria?
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Cystathionine Synthase
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AA transport disease with build up leading to kidney stones ?
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Cystinuria
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In ETC what is enzyme of complex 3?
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Cytochrome bc
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In ETC what is enzyme of complex 4?
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Cytochrome C oxidase
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What enzyme is active during oxidative deamination phase of amino acid breakdown?
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dehydrogenase
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What cofactor is PDH?
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E1
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What co factor is Dihydrolipoyl transacetylase?
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E2
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What co factor is Dihydrolipoyl dehydrogenase?
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E3
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What cleaves sugar at inner bonds and example?
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Endoglycosidase, a-amylase
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What activates Trypsin?
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enteropeptidase
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What cleaves sugar at end and example?
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Exoglycosidase, Maltase
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What activates pyruvate kinase?
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F1,6 bisphosphate
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What activates PKF-1 ?
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F2,6-bisphosphate, AMP
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What 2 things are turned into Acetyl-CoA?
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Fatty acyl-CoA, Pyruvate
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What is the rate limiting step in heme synthesis?
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first, ALA synthase
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What binds up Glucokinase to inactivate it?
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GKRP (Glucokinase regulatory protein)
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What is pkA activated by ?
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Glucagon
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What enzyme in liver that prevents hyperglucemia ?
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Glucokinase
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What does glucose first turn into to trap in cell?
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Glucose 6-P
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Glc uptake in liver and pancreatic B cells
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GLUT 2
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Glc uptake in muscles and adipose tissue, insulin dependent
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GLUT 4
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In transmination moves NH3+ from amino acid onto a-ketoglutarate forming what for transport from tissues to liver?
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Glutamate
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What enzyme frees NH4+ from glutamate?
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Glutamate dehydrogenase
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AA biosynthesis a-ketoglutarate + NH4 to glutamate via?
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glutamate dehydrogenase
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De Novo purine synthesis rate limiting step enzyme?
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Glutamine Phosphoribosyl pyrophosphate aminotransferase
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Non-enzymatic addition of glucose to proteins
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Glycation
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What are the two shuttles that allow NADH to go from cytosol to mitochondria?
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Glycerol 3-phosphate, malate-aspartate
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Salvage purine synthesis pathway for making GMP?
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Guanine + PRPP by hypoxanthine guanine PRT
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AA transport disease effecting trytophan uptake?
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Hartnup
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What are the two enzymes that make Glucose 6-P
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Hexokinase (low Km), Glucokinase (high Km)
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What is Glucokinase inhibited by?
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high F6P
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What is Glucokinase activated by?
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high glucose
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What does Lesch-Nyhan result in, intermediate component levels
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High PRPP, Low IMP, low GMP
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What does Benzoate form to remove ammonia group?
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Hippurate
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Salvage purine synthesis pathway for making IMP?
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Hypoxanthine + PRPP by hypoxanthine guanine PRT
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Where does carboxypeptidase B cleave (after double bond)?
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If C terminal Arg or Lys
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Activation of PDH kinase does what to PDH complex in turning pyruvate to Acetyl CoA?
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Inactivate
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In ETC mitochondria where is H+ concentration higher?
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intermembrane space
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What cleaves sugar at end branches?
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Isomaltase
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What are the two groups of amino acids based on their products of catabolism?
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Ketogenic, Glucogenic
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Purine salvage deficiency with HGPRT?
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Lesch-Nyhan syndrome
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What are only ketogenic aa's?
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Leucine, Lysine
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Where are de novo synthesized nucleotides degraded?
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liver
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What activates pepsinogen?
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low pH
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How is Glycerol 3-phosphate used in mitochondria for ETC?
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making FADH2
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How is malate-aspartate used in mitochondria for ETC?
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making NADH
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What is the thermodynamically unfavorable reaction enzyme of the Kerb's Cycle (TCA)?
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Malate Dehydrogenase
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Deficiency in BCKAD (branched chain a-keto acid dehydrogenase complex)?
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Maple Syrup urine disease
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what is aa cyst made from?
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Met
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What is responsible for fast urea cycle initiation?
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NAC (N-acetyl glutamate)
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De Novo purine synthesis - what is added to turn IMP to GMP?
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NAD+, Gln, ATP
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In ETC what is enzyme of complex 1?
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NADH dehydrogenase
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What does the Kreb's cycle yeild?
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NADH, FADH2, CO2
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What moves back across membrane seperating into mito matix from Cytosol in Urea Cycle?
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Ornithine
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What makes OMP?
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Orotate + PRPP
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What is the most common defect to the urea cycle?
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OTC (ornithine transcarbamylase)
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What changes F6P to Fructose 1,6-bisphsphate?
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PFK-1
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What causes activity increase of F2,6-bisposphate
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PFK-2
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What is aa tyr made from?
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Phe
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What is the main defect cause of Phenylketouria (PKU)?
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Phenylalanine hydroxylase
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What inhibits Pyruvate Kinase?
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pkA
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In transmination moves NH3+ from amino acid onto a-ketoglutarate via what COENZYME?
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PLP (pyridoxal phosphate)
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What is inhibited by lead in heme synthesis?
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Porphobilinogen via ALA dehydratase, Heme via ferrochelatase
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Inability to make Uroporphyrinogen decarboxylase?
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Porphyria cutanea tarda
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What kind of nitrogen balance is intake > excretion ?
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positive nitrogen balance
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What are 2 drugs used for gout (hyperuricemia) - uric acid overproducters?
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Allopurinol, Febuxostat
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De Novo purine synthesis rate limiting step?
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PRPP to IMP
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What has both De novo and salvage nucleotide synthesis?
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Purine
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stuff added to PRPP for nucleotide synthesis ?
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Purine
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What are the essential amino acids?
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PVT TIM HALL
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build base then add PRPP at end for nucleotide synthesis?
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Pyrimidine
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What is OMP an intermediate for?
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Pyrimidine synthesis
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What turns Phosphoenolpyruvate to pyruvate?
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Pyruvate kinase
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What regulation is feed forward?
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Pyruvate kinase
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What inhibits ETC at first stage after NADH?
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Rotenone
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Where are ingested nucleotides degrated ?
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small intestines
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Where is rate limiting step in pyrimidine synthesis?
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Step 1 - In building Orotate from Glutamine
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In ETC what is enzyme of complex 2?
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Succinate dehydrogenase
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Both Glutamine and Asparagine are made by ?
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Synthetase
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What are the two ways of transfering 1 C?
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Tetrahydrofolate, SAM (S-adenosylmethionine)
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What does Methotrexate (Folic analogs) inhibit?
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TH4 (Tetrahydrofolate), in purine synthesis
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What activates chymotrypsin?
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trypsin
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What activates Procaroxypeptidase?
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trypsin
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What activates Proelastase?
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trypsin
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Where does chymotrypsin cleave (after double bond)?
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Tyr, Phe, Trp
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What is Phenylalanine suppose to covert to using BH4 and O2?
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Tyrosine
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What does a low Respirator Control Ratio signify?
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uncoupling, proton leak
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What is the main test for OTC?
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Urine orotic acid levels
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Last purine intermediate before uric acid excretion?
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Xanthine
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