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10 Cards in this Set
- Front
- Back
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cytosolic HMG -CoA>mevalonate (cholesterol syn),cholesterol and bile salts -,insulin +, use statins to inhibit activity in tx of high cholesterol
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HMG CoA reductase
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homocysteine>methionine (B12),homocystinuria(mental retardation,osteoporosis,lens dislocation,CVD)
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Homocysteine Methyl Transferase (methionine synthetase)
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methylmalonyl CoA>succinyl CoA (B12),propionic acid pathway:Valine,isoleucine,methionine,threonine and odd chain FA, neonatal ketoacidosis
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Methylmalonyl CoA mutase
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bilirubin(uncon/indir)>biliruben diglucuronide(con/dir)(liver), inc. uncon/indir:jaundice, kernicterus, conditions: hemolysis, Crigler-Najjar, Gilbert's, physiologic jaundice(newborns)
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UDP-Glucuronyl transferase
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folate>DHF>THF (purine/thymidine syn), Folate def:Megaloblastic anemia,homocystinemia Methotrexate (antineoplastic) -
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Dihydrofolate reductase
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hypoxanthine>xanthine(molybdenum),xanthine>uric acid (purine syn),inc: gout,allopurinol -
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Xanthine oxidase
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cotransitional modification introduces Ca+ binding sites on Ca dependent proteins (factors II,VII,IX,X, C & S)through gamma carboxylation of glutamyl residues (Vitamin K dependent)
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Gamma-glutyl carboxylase
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pyruvate>acetyl CoA (thiamine dependent) insulin+, alcoholics thiamine deficient=lactic acidosis bc no acetyl CoA
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Pyruvate Dehydrogenase
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rate limiting enzyme, insulin & NADP +,NADPH -, regulates NADPH produced in PPP, NADPH = biosynthesis FA, chol, and nucleotide, kills bacteria(PMN), and removes free radicals(Glutathione dependent), hemolytic anemia, heinz body
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G6PD
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sphingolipid catabolism, Tay-Sachs(inc ganglioside GM2)=cherry red spots, blindness, psychomotor retardation, death <2yrs
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Hexominadase A
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