Description

Glutaryl-CoA dehydrogenase (GCDH) is an acyl dehydrogenase involved in the metabolism of lysine, hydroxylysine, and tryptophan. The active enzyme exists as a homotetramer in the mitochondrial matrix. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I, an inherited disorder in which the body is unable to process certain proteins properly. Excessive levels of amino acids and their intermediate breakdown products can accumulate and cause damage to the brain (Hedlund et al., 2006).

External Information

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