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(C4) Butyrylcarnitine

目录号 数量 价格 提前通知时间
4266-1mg 1 mg 请查询 7 天
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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.

General properties

Appearance: white solid
Molecular weight: 267.75
CAS number: 162067-50-7 (chloride), 25576-40-3 (inner salt)
Molecular formula: C11H22ClNO4
Solubility: DMSO, DMF, ethanol
Quality control: NMR 1H and HPLC-MS (95+%)
Storage conditions: 24 months after receival at -20°C in the dark. Transportation: at room temperature for up to 3 weeks. Desiccate.
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