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Protein turnover . Catabolism of amino acids III

Protein turnover . Catabolism of amino acids III. István Léránt. CATABOLISM OF AMINO ACIDS ‚C’. Phe Tyr Trp Role of molecular oxygen. CATABOLISM OF AMINO ACIDS ‚C’. Phe Tyr Trp Role of molecular oxygen. CATABOLISM OF AMINO ACIDS ‚C’. Phenylketonuria. Phe Typ Trp.

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Protein turnover . Catabolism of amino acids III

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  1. Protein turnover.Catabolism of aminoacids III István Léránt

  2. CATABOLISM OF AMINO ACIDS‚C’ Phe Tyr Trp Role of molecularoxygen

  3. CATABOLISM OF AMINO ACIDS‚C’ Phe Tyr Trp Role of molecularoxygen

  4. CATABOLISM OF AMINO ACIDS‚C’ Phenylketonuria Phe Typ Trp

  5. CATABOLISM OF AMINO ACIDS‚C’ • Phenylketonury • Hyperphenylalaninaemia I: phenylalanin-hydroxylase • Dihydrobiopterin-reductase (hyperphenylalanaemia II, III) • Dihydrobiopterinbiosynthesis (hiperphenylalaninaemia IV, V) Phe Tyr Trp

  6. CATABOLISM OF AMINO ACIDS‚C’ Phe Tyr Trp

  7. CATABOLISM OF AMINO ACIDS‚C’ p-hidroxifenilpiruváthidroxiláz Phe Tyr Trp Transzamináz aktivitás Homogentizinsavoxidáz Tyrosine-transaminase: Type II tyrosinemia Neonataltyrosinemia: p-OH-phenyl-pyruvatehydroxylase Alkaptonuria: homogentisateoxydase Fumarylacetoacetatehydroxylase: Type I tyrosinemia

  8. CATABOLISM OF AMINO ACIDS‚C’ • Tyrosinaemia Type I: • Genetical defect of: • fumaryloacetoacetate and maleyloacetoacetatehydrolase • [Tyr]plasma 6-12 mg/dl, [Met] á • Acuttyrosinosis: „cabbage odor”, diarrhea, vomit, death (at age 6-8 months) • Chronic tyrosinaemy: milder symptoms, death • (at age 10 years) Phe Tyr Trp

  9. CATABOLISM OF AMINO ACIDS‚C’ • Tyrosinaemiatype II: • Genetical defect of: tyrosinetransaminase • [Tyr]plasma 4-5 mg/dl • Eye and skinlesions • Mentalretardation • Neonataltyrosinaemia: • Relativelack of p-hydroxy-phenylpyruvate-hydroxylase • Protein poordiet Phe Tyr Trp

  10. CATABOLISM OF AMINO ACIDS‚C’ • Alcaptonuria • Homogentisinicoxydase • 1859 – Theory of Garrod elmélet • Darkurinemayoccur • Oochronosis Phe Tyr Trp

  11. NIACIN (B3) CATABOLISM OF AMINO ACIDS‚C’ Phe Tyr Trp Catabolism of tryptophane Trpà Nicotinamide Tryptophaneoxygenase (inducible) Kinurenineformilase Kinureninehydroxylase Kinureninase (PLP) KINURENIN àNiacin (Vitamin B3)

  12. CATABOLISM OF AMINO ACIDS‚C’ • Vitamin B6 deficiency: • Kinurenine is converted into xanturenate – urine • Synthesis of NAD+ & NADP+â • Synthesis of pyrimidineâ • Niacin deficiency: • pellagra • Hartnup-disease: • A family of London (19th century) • Trp (neutral) amino acid • Disorder of intestinal and renal transport Phe Tyr Trp

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