The Study of Organic Acids Metabolism in a Patient with Ornithine Transcarbamylase (OTC) Deficiency
The formation of glutamate from ammonia and α-ketoglutarate (α-KG) is one of major pathways to remove excessive ammonia. This pathway is especially important in the urea cycle enzymopathies. It has been reported that the plasma α-KG concentration was increased in the patients with chronic hepatic dysfunctions, Reye’s syndrome and fulminent hepatic failure1,2. On the other hand, Batshaw et al.3 reported that plasma α-KG level was decreased in a hyperammonemic state in the patients with various urea cycle enzymopathies. In the patients with urea cycle enzymopathies, the citric acid cycle (TCA cycle) which is closely linked to the urea cycle, might be significantly influenced due to excessive ammonia. Thus it seems important to study the changes of the TCA cycle components in the urea cycle enzymopathy.
KeywordsGlutamate Dehydrogenase Urea Cycle Plasma Ammonium Excessive Ammonia Ornithine TRANSCARBAMYLASE
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