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The a -amino group

The a -amino group. Tr ansamination Exception Pro Hyp Thr Lys. Catabolism of carbonic skeleton of amino acids. Medical importance: Disease - low frequency Mental retardation Prenatal diagnosis Postnatal diagnosis - treatment. Catabolism of carbon skeleton. Ala, Cys, Gly, Hyp,

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The a -amino group

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  1. Thea-amino group • Transamination • Exception • Pro • Hyp • Thr • Lys

  2. Catabolism of carbonic skeleton of amino acids • Medical importance: • Disease - low frequency • Mental retardation • Prenatal diagnosis • Postnatal diagnosis - treatment

  3. Catabolism of carbon skeleton

  4. Ala, Cys, Gly, Hyp, Ser, Thr Arg, His Gln, Pro l-Glutamate Ile, Leu,Trp Pyruvate a-Ketoglutarate Ile, Met, Val Citrate Acetyl-CoA Succinyl-CoA Acetoacetyl-CoA Citrat cyclus Oxalacetat Leu, Lys, Phe, Trp, Tyr Fumarat Tyr, Phe l-Aspartat l-Asn

  5. Asn, Asp - Oxaloacetate • Asparaginase • Transaminase

  6. Ala, Cys, Gly, Hyp, Ser, Thr Arg, His Gln, Pro l-Glutamate Ile, Leu,Trp Pyruvate a-Ketoglutarate Ile, Met, Val Citrate Acetyl-CoA Succinyl-CoA Acetoacetyl-CoA Citrat cyclus Oxalacetat Leu, Lys, Phe, Trp, Tyr Fumarat Tyr, Phe l-Aspartat l-Asn

  7. Gln & glu a-ketoglutarate • Glutaminase • Transaminase

  8. Pro -a-ketoglutarate Proline Arginine L-glutamat-g-semialdehyd L-Glutamat a-ketoglutarate

  9. Pro -a-ketoglutarate (1)

  10. Pro -a-ketoglutarate (2)

  11. Pro -a-ketoglutarate • 2 autosomal recessive hyperprolinemia • Hyperprolinaemia I: prolin-dehydrogense (Hz –symptoms of hyperprolinaemia) • Hyperprolinaemia II: Glutamate-g-semialdehyd dehydrogenase (hyperhydroxypolinaemia, Hz absence of symptoms of hyperprolinaemia)

  12. Arg & ornitin - a-ketoglutarate

  13. Arg & ornitin - a-ketoglutarate • The defect of ornitin d-aminotransferase results in: • [ornitin] is enhanced, blindness. • Hyperornitinaemia – hyperammonaemia syndrom: [ornitin]plazmais enhanced.Lowered mitochondrial transport.

  14. L-His -a-ketoglutarate

  15. L-His - a-ketoglutarate

  16. L-His - a-ketoglutarate • Histininaemia • Histidase enzyme defect: 1:11500 • [His]blood, urineis elevated • Typical impediment in speech, benign syndrome • Urokaninic aciduria • Autosomal recessiveinheritance • [His]urineis elevated • benign syndrome

  17. Ala, Cys, Gly, Hyp, Ser, Thr Arg, His Gln, Pro l-Glutamate Ile, Leu,Trp Pyruvate a-Ketoglutarate Ile, Met, Val Citrate Acetyl-CoA Succinyl-CoA Acetoacetyl-CoA Citrat cyclus Oxalacetat Leu, Lys, Phe, Trp, Tyr Fumarat Tyr, Phe l-Aspartat l-Asn

  18. Amino acids - pyruvate L-Threonin L-Glycine Cystine L-Serin Pyruvat L-Cystein L-Alanine Piruvat-dehydrogenase Acetyl-CoA

  19. L-Glycin • Synthesis of glutathion, creatin, purine skeleton, conjugated bile acids, hem • Glucoplastic aminoacid • Catabolism of Gly: • Glycin – Serin – Pyruvate – Acetyl-CoA • Glycine cleavage – Glycine synthase complex

  20. L-Glycin Serin hydroxymethyl transferase

  21. L-Glycin Cleavage of Gly by mitochondrial glycine synthase complex

  22. L-Glycin • Glycinuria: • 0,6 – 1 g glycin/day • Oxalate-typ neprolyth • Defect in tubular reabsorption of kidneys • Primary hyperoxaluria • Glycin deamination – glyoxilate, oxalate • Ca-oxalate type neprolyths

  23. Amino acids - pyruvate L-Threonin L-Glycine Cystine L-Serin Piruvate L-Cysteine L-Alanin Acetyl-CoA

  24. Amino acids - pyruvate L-Threonin L-Glycin Cystine L-Serin Piruvat L-Cysteine L-Alanin Acetyl-CoA

  25. Cystin – cystein conversion

  26. Cystein – pyruvate conversion:2 ways • 2 ways • Direct oxydative: cystein sulfinate • Transamination: 3-merkaptopyruvate • „activ” sulfate formation (3’-phosphoadenosine-5’-phosphosulfate) • Glutathion syntesis

  27. Cystein – pyruvate conversion:(i) direct oxydative way • Cystein dioxygenase • Fe2+ • NAD(P)H • Desulfinase / spontan reaction Taurine

  28. Cystein – pyruvate conversion :(ii) transamination • 3-merkapto-lactate – in human urin • ~ + cysteinnel disulfid – in urine • [merkaptolactat – cystein]urine merkaptolactate – in cystein disulfid uria

  29. 3'-phosphoadenosine-5'-phosphosulfate, (PAPS).

  30. Amino acids - pyruvate L-Threonin L-Glycin CO2 + NH4+ Cystine L-Serin Piruvat L-Cysteine L-Alanin Acetyl-CoA

  31. Treonin: treonin aldolaseètwo ways Acetaldehyde threonin aldolase Glycineè Methylen H4 folate +CO2+NH4+ L-serine Piruvate

  32. Glyoxylate is formed from 4-hydroxyprolin képződik

  33. 4-hydroxyprolin piruvate& glyoxylate

  34. 4-hydroxyprolin piruvate& glyoxylate • Hyperhydroxyprolinaemia: • Hydroxyproline dehydrogenase • [4-hydroxyproline]plasma • Autosomal resessive trait • Glutamate-g-semialdehyde dehydrogenase • L-D1-Pyrroline-3-hydroxi-5-carboxilate • Lack of hyperprolinaemia

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