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Biosynthesis of the Nutritionally Nonessential Amino Acids

Biosynthesis of the Nutritionally Nonessential Amino Acids. BIOMEDICAL IMPORTANCE. Amino acids present in proteins Amino acid deficiency Kwashiorkor Marasmus Caloric intake & specific amino acids are deficient

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Biosynthesis of the Nutritionally Nonessential Amino Acids

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  1. Biosynthesis of the NutritionallyNonessential Amino Acids

  2. BIOMEDICAL IMPORTANCE • Amino acids present in proteins • Amino acid deficiency • Kwashiorkor • Marasmus • Caloric intake & specific amino acids are deficient • Humans can synthesize 12 of the 20 common amino acids from the amphibolic intermediates

  3. Amino acid requirements of humans

  4. amino acids from • Amphibolic intermediates • α-ketoglutarate • Oxaloacetate • 3-phosphoglycerate • Dietary amino acids

  5. The glutamate dehydrogenase reaction

  6. Formation of alanine by transamination of pyruvate

  7. The asparagine synthetase reaction

  8. The glutamine synthetase reaction

  9. Serine biosynthesis

  10. Formation of glycine from choline

  11. The serine hydroxymethyltransferase reaction

  12. Biosynthesis of proline from glutamate

  13. Conversion of homocysteine and serine to homoserine and cysteine

  14. The phenylalanine hydroxylase reaction

  15. The prolyl hydroxylase reaction

  16. Selenocysteine (top) and the reaction catalyzed by selenophosphate synthetase (bottom)

  17. Selenocysteine-specific UGA codon • Selenocysteine insertion element

  18. Catabolism of proteins • Urea synthesis

  19. Partial reactions in the attachment of ubiquitin (UB) to proteins.

  20. Overall flow of nitrogen in amino acid catabolism

  21. Transamination

  22. Alanine aminotransferase (top) and glutamate aminotransferase (bottom).

  23. The L-glutamate dehydrogenase reaction

  24. Oxidative deamination catalyzed by L-amino acid oxidase

  25. The glutamine synthase reaction

  26. The glutaminase reaction

  27. Reactions and intermediates of urea biosynthesis

  28. METABOLIC DISORDERS • Hyperammonemia Type 1. • Hyperammonemia Type 2 • Citrullinemia • argininosuccinate synthase • Argininosuccinicaciduria • Argininosuccinase • Hyperargininemia

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