Biochemistry - top enzymes
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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
- HMG CoA reductase
- homocysteine>methionine (B12),homocystinuria(mental retardation,osteoporosis,lens dislocation,CVD)
- Homocysteine Methyl Transferase (methionine synthetase)
- methylmalonyl CoA>succinyl CoA (B12),propionic acid pathway:Valine,isoleucine,methionine,threonine and odd chain FA, neonatal ketoacidosis
- Methylmalonyl CoA mutase
- bilirubin(uncon/indir)>biliruben diglucuronide(con/dir)(liver), inc. uncon/indir:jaundice, kernicterus, conditions: hemolysis, Crigler-Najjar, Gilbert's, physiologic jaundice(newborns)
- UDP-Glucuronyl transferase
- folate>DHF>THF (purine/thymidine syn), Folate def:Megaloblastic anemia,homocystinemia Methotrexate (antineoplastic) -
- Dihydrofolate reductase
- hypoxanthine>xanthine(molybdenum),xanthine>uric acid (purine syn),inc: gout,allopurinol -
- Xanthine oxidase
- 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)
- Gamma-glutyl carboxylase
- pyruvate>acetyl CoA (thiamine dependent) insulin+, alcoholics thiamine deficient=lactic acidosis bc no acetyl CoA
- Pyruvate Dehydrogenase
- 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
- G6PD
- sphingolipid catabolism, Tay-Sachs(inc ganglioside GM2)=cherry red spots, blindness, psychomotor retardation, death <2yrs
- Hexominadase A