Le figure riportate in questo CD sono state riprodotte

1. Le figure riportate in questo CD sono state riprodotte dal testo “I principi di Biochimica di Nelson-COX” o da altro materiale didattico disponibile in rete. Il CD deve essere usato per consultazione ed è vietato riprodurlo o commercializzarlo. Il CD deve essere riconsegnato al Prof. Guarnieri al termine della consultazione.

16. Piridossamina-5 P Piridossale-5 P Alfa-chetoglutarico Glutamico

30. Urea Cycle Defects • HYPERAMMONEMIAS • Carbamoyl Phosphate Synthetase I (CPS-I) Deficiency • Ornithine Transcarbamylase (OTC) Deficiency • N-Acetylglutamate Synthetase Deficiency • Argininosuccinic Aciduria   - Argininosuccinate Lyase Deficiency • Hyperargininemia   - Arginase Deficiency • Citrullinemia   - Argininosuccinate Synthetase Deficiency • Ornithine Aminotransferase Deficiency

31. The Glutamine Synthase Reaction • The reaction catalyzed by glutamine synthetase is: • glutamate + NH4+ + ATP -------> glutamine + ADP + Pi + H+ • The glutamine synthetase reaction is also important in several respects. First it produces glutamine, one of the 20 major amino acids. Second, in animals, glutamine is the major amino acid found in the circulatory system. Its role there is to carry ammonia to and from various tissues but principally from peripheral tissues to the kidney, where the amide nitrogen is hydrolyzed by the enzyme glutaminase (reaction below); this process regenerates glutamate and free ammonium ion, which is excreted in the urine. • glutamine + H2O -------> glutamate + NH3 • Note that, in this function, ammonia arising in peripheral tissue is carried in a nonionizable form which has none of the neurotoxic or alkalosis-generating properties of free ammonia.