Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I.
2001
Glutaric aciduria type I (GA-I) is an autosomal recessive disorder of lysine, hydroxylysine and tryptophan metabolism which results in an accumulation and increased excretion of glutaric acid and its metabolites [1]. Glutaryl-coenzyme A dehydrogenase (GCDH) is a multifunctional enzyme responsible for the dehydrogenation and decarboxylation of glutaryl-CoA to crotonyl-CoA in the degradative pathway of L-lysine, L-hydroxylysine, [...]
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