Glutaroyl carnitine

Chemical Properties

Clear Thick Gel

Uses

A metabolite of L-Carnitine (C184110).

Definition

ChEBI: O-glutaroyl-L-carnitine is an O-acyl-L-carnitine in which the acyl group is specified as glutaroyl. It has a role as a human metabolite. It is functionally related to a glutaric acid.

Biochem/physiol Actions

Increased formation and excretion of glutarylcarnitin results from a glutaryl-CoA dehydrogenase deficiency, an inborn error of lysine and tryptophan metabolism. Secondary carnitine depletion due to increased formation and urinary excretion of glutarylcarnitine is suggested to play an important role in the neuropathogenesis of glutaryl-CoA dehydrogenase deficiency, inducing excitotoxic neuronal damage and mitochondrial dysfunction.