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Download Biosynthesis of the Nutritionally Nonessential Amino Acids
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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 • Humans can synthesize 12 of the 20 common amino acids from the amphibolic intermediates Amino acid requirements of humans • amino acids from – Amphibolic intermediates • α-ketoglutarate • Oxaloacetate • 3-phosphoglycerate – Dietary amino acids The glutamate dehydrogenase reaction Formation of alanine by transamination of pyruvate The asparagine synthetase reaction The glutamine synthetase reaction Serine biosynthesis Formation of glycine from choline The serine hydroxymethyltransferase reaction Biosynthesis of proline from glutamate Conversion of homocysteine and serine to homoserine and cysteine The phenylalanine hydroxylase reaction The prolyl hydroxylase reaction Selenocysteine (top) and the reaction catalyzed by selenophosphate synthetase (bottom) • Selenocysteine-specific UGA codon • Selenocysteine insertion element Catabolism of proteins • Urea synthesis Partial reactions in the attachment of ubiquitin (UB) to proteins. Overall flow of nitrogen in amino acid catabolism Transamination Alanine aminotransferase (top) and glutamate aminotransferase (bottom). The L-glutamate dehydrogenase reaction Oxidative deamination catalyzed by L-amino acid oxidase The glutamine synthase reaction The glutaminase reaction Reactions and intermediates of urea biosynthesis METABOLIC DISORDERS • Hyperammonemia Type 1. • Hyperammonemia Type 2 • Citrullinemia – argininosuccinate synthase • Argininosuccinicaciduria – Argininosuccinase • Hyperargininemia
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