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The role of glutarylcarnitine in the body, like that of other acylcarnitines, is in facilitating the transport of fatty acids into mitochondria for beta-oxidation, therefore glutarylcarnitine is used in the diagnosis of metabolic disorders.

The product is used primarily as a control for MS/MS.

Glutarylcarnitine has a wide range of potential uses in both research and clinical applications. Glutarylcarnitine and carnosylcarnitine may be biomarkers for the diagnosis of autism spectrum disorders. Glutarylcarnitine has been studied as a potential therapeutic agent in the treatment of diabetes, metabolic syndrome, and obesity.

Clear Thick Gel

A metabolite of L-Carnitine (C184110).

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.

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.

Human Endogenous Metabolite

Store at -20°C