Recent advances in amino acid and organic acid metabolism.
This paper focuses on the three areas in this field in which there have been advances in amino acid and organic acid metabolism. These are the description of glutamine synthetase deficiency, the elucidation of the mechanism of pyridoxine-dependent convulsions, and a hypothesis to explain the neurological complications of some organic acidaemias.
Leonard
J V
JV
Biochemistry, Endocrinology and Metabolism Unit, Institute of Child Health, University College London, London, UK. J.Leonard@ich.ucl.ac.uk
D000143:Acids / Q000097:blood / Q000378:metabolism*; D000596:Amino Acids / Q000378:metabolism*; D000818:Animals; D001671:Biochemistry / Q000639:trends*; D005974:Glutamate-Ammonia Ligase / Q000172:deficiency; D006801:Humans; D009422:Nervous System Diseases / Q000209:etiology; D011736:Pyridoxine / Q000378:metabolism / Q000627:therapeutic use; D012640:Seizures / Q000209:etiology; D014803:Vitamin B Complex / Q000378:metabolism / Q000627:therapeutic use
D000143:Acids; D000596:Amino Acids; D014803:Vitamin B Complex; D005974:Glutamate-Ammonia Ligase; D011736:Pyridoxine