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Organic acidaemia and hyperammonaemia: Review
Author(s) -
Walser M.,
Stewart P. M.
Publication year - 1981
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf02263648
Subject(s) - medicine
Recent clinical and experimental evidence on the effects of organic acids in producing or ameliorating hyperammonaemia is reviewed. The importance of hepatic mitochondrial N ‐acetylglutamate and its precursors, glutamate and acetyl‐CoA, in the control of ureagenesis and thus blood ammonia levels is emphasized by recent work. The hypothesis is proposed that protein loads stimulate urea cycle activity via glutamate‐induced changes in N ‐acetylglutamate concentration, while the effects of organic acids on ureagenesis are related in a predictable way to their effects on hepatic concentrations of acetyl‐CoA.