(C4) Butyrylcarnitine (4266-1mg)

Butyrylcarnitine, also known as (3R)-3-(butyryloxy)-4-(trimethylammonio)butanoate or L-carnitine butyryl ester, is classified as a member of the acylcarnitines. Acylcarnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Butyrylcarnitine may be used as an analytical reference standard for the separation and identification of underivatized butyryl-L-carnitine in human plasma samples using high-performance liquid chromatography coupled to tandem mass spectrometry (HPLC-MS/MS). Butyrylcarnitine can be used to study its role in energy production and its metabolic effects, to understand metabolic diseases and conditions associated with mitochondrial dysfunction. Butyrylcarnitine is a major biological marker of short-chain acyl-CoA dehydrogenase deficiency, glutaric acidemia type II and isobutyrylglycinuria, and ethylmalonic encephalopathy. The product is used primarily as a control for MS/MS.