L-Isovalerylcarnitine [(2R)-3-carboxy-2-(3-methylbutanoyloxy)propyl]-trimethylazanium chloride
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Isovaleryl-L-carnitine is a naturally occurring acylcarnitine metabolized from leucine via a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase, which results in an accumulation of derivatives of isovaleryl-CoA.
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