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Protein turnover Catabolism of amino acids II

Protein turnover Catabolism of amino acids II. István Léránt. CATABOLISM OF AMINO ACIDS ‚C’. Pyruvate Acetyl-CoA Acetoacetyl-CoA Alpha-ketoglutarate Succunyl-CoA Fumarate Oxalacetate. CATABOLISM OF AMINO ACIDS ‚C’. Ketoplastic amino acids Acetyl-CoA Acetoacetyl -CoA

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Protein turnover Catabolism of amino acids II

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  1. Protein turnoverCatabolism of aminoacids II István Léránt

  2. CATABOLISM OF AMINO ACIDS ‚C’ Pyruvate Acetyl-CoA Acetoacetyl-CoA Alpha-ketoglutarate Succunyl-CoA Fumarate Oxalacetate Protein turnover. Catabolism of amino acids

  3. CATABOLISM OF AMINO ACIDS ‚C’ • Ketoplastic amino acids • Acetyl-CoA • Acetoacetyl-CoA • Glucoplasticamino acids • Pyruvate • Alpha-ketoglutarate • Succinyl-CoA • Fumarate • Oxalacetate • Gluco- and ketoplastic amino acids Pyruvate Acetyl-CoA Acetoacetyl-CoA Alpha-ketoglutarate Succunyl-CoA Fumarate Oxalacetate Protein turnover. Catabolism of amino acids

  4. Protein turnover. Catabolism of amino acids

  5. Protein turnover. Catabolism of amino acids

  6. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Ala Ser Cys Thr results in pyruvateformation Protein turnover. Catabolism of amino acids

  7. Carbonskeleton is degradedtopyruvate L-Treonine L-Glycine CO2 + NH4+ Cystine L-Serine Pyruvate L-Cysteine L-Alanine Pyruvate-dehydrogenase Acetil-KoA Protein turnover. Catabolism of amino acids

  8. Protein turnover. Catabolism of amino acids

  9. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Asp Asn Results in oxaloacetate L-aspartatea-ketoglutarateL-glutamateoxaloacetate Protein turnover. Catabolism of amino acids

  10. Protein turnover. Catabolism of amino acids

  11. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Gln Glu Pro Arg His Results in formation of a-ketoglutarate Protein turnover. Catabolism of amino acids

  12. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Gln Glu Pro Arg His results in a-ketoglutarate His – precurcor Histamine (biogenamine) Carnosine (dipeptide, brain, muscle) Protein turnover. Catabolism of aminoacids

  13. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Gln Glu Pro Arg His results in a-ketoglutarate His – precurcor Histamine (biogenamine) Carnosine (dipeptide, brain, muscle) Histidinaemia, histidinuria Histidase deficiency [histamine] á, [imidazole] á In blood, urine, cerebrospinal fluid Decreased [urocanic acid] Rare, autosomal recessive Asymptomatic, later hyperactivity, impeded speach Protein turnover. Catabolism of amino acids

  14. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Gln Glu Pro Arg His results in a-ketoglutarate His – precursor of Histamine Histamine (biogenamine) Carnosine (dipeptide, brain, muscle) Folicacid Laboratory test in folicaciddeficiency Figlu (Form-imino-glutamate) á Protein turnover. Catabolism of amino acids

  15. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Gln Glu Pro Arg His results in a-ketoglutarate Protein turnover. Catabolism of amino acids

  16. CATABOLISM OF AMINO ACIDS ‚C’ Degradation of Gln Glu Pro Arg His results in a-ketoglutarate • Prolineoxydase, pyrroline-5-carboxylate dehydrogenase • Hyperprolinaemia I • Hyperprolinaemia I:prolin-dehydrogenase • (Hz –symptoms of hyperprolinaemia) • Hyperprolinaemia II • Hyperprolinaemia II:Glutamate-g-semialdehyddehydrogenase • (hyperhydroxypolinaemia, Hz absence of symptomsofhyperprolinaemia) • , Protein turnover. Catabolism of amino acids

  17. Protein turnover. Catabolism of amino acids

  18. CATABOLISM OF AMINO ACIDS ‚C’ Catabolism results in formation of succinyl-CoA Met Val Ile ß-oxidation of fatty acids of odd-numberedchain Cholesterol – bile acids Biotin Vit B12 Protein turnover. Catabolism of amino acids

  19. Met a-ketobutyrate CATABOLISM OF AMINO ACIDS ‚C’ Val Propionyl-CoA Methylmalonyl-CoA Leu Catabolism results in formation of succinyl-CoA Met Val Ile Succinyl-CoA Protein turnover. Catabolism of amino acids

  20. CATABOLISM OF AMINO ACIDS ‚C’ Catabolism results in formation of succinyl-CoA Met Val Ile Protein turnover. Catabolism of amino acids

  21. CATABOLISM OF AMINO ACIDS ‚C’ • Hyperhomocysteinemia/hyperhomocysteinaemia [homocysteine] bloodáá • Pyridoxine/ (B6) • folic acid (B9) • Vitamin B12 • Deficiency of • methylentetrahydrofolatereductasemethioninsynthetase • Riskfactorforrecurrentvenousthrombosis Met Val Ile Protein turnover. Catabolism of amino acids

  22. CATABOLISM OF AMINO ACIDS ‚C’ Leu Val Ile Transaminaseactivity a-ketoacid-dehydrogenase a-ketoacidderivative CO2 + Acyl-CoAtioester a,ß-unsaturatedacyl-CoAthioester ß-Hydroxymethyl- Glutaryl-CoA Leu Propionyl-CoA + Acetyl-Coa Ile Succinyl-CoA Val Protein turnover. Catabolism of amino acids

  23. CATABOLISM OF AMINO ACIDS ‚C’ Branchedalpha-ketoaciddehydrogenasemultienzymcomplex Alpha-ketoglutaratedehydrogenase PDH PDH (MapleSyrupUrineDisease) Methylmalonicaciduria Propionicacidemia Protein turnover. Catabolism of amino acids

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