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2-Methylbutyryl-L-carnitine-d3(chloride)
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
2-Methylbutyryl-L-carnitine-d3(chloride)图片
规格:98%
分子量:284.8
包装与价格:
包装价格(元)
500ug电议
1mg电议
5mg电议

产品介绍
A neuropeptide with diverse biological activities
货号:ajcx21274
CAS:N/A
分子式:C12H21D3NO4.Cl
分子量:284.8
溶解度:DMF: 15 mg/ml,DMSO: 20 mg/ml,Ethanol: 25 mg/ml,PBS (pH 7.2): 10 mg/ml
纯度:98%
存储:Store at -20°C
库存:现货

Background:

2-Methylbutyryl-L-carnitine-d3is intended for use as an internal standard for the quantification of 2-methylbutyryl-L-carnitine by GC- or LC-MS. 2-Methylbutyryl-carnitine is a naturally occurring acylcarnitine that is producedviaL-isoleucine metabolism.1Plasma levels of 2-methylbutyryl-carnitine are elevated in patients with non-alcoholic steatohepatitis (NASH).2Elevated levels of 2-methylbutyryl-carnitine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency.3


1.Gibson, K.M., Burlingame, T.G., Hogema, B., et al.2-Methylbutyryl-coenzyme A dehydrogenase deficiency: A new inborn error of L-isoleucine metabolismPediatr. Res.47(6)830-833(2000) 2.Kalhan, S.C., Guo, L., Edmison, J., et al.Plasma metabolomic profile in nonalcoholic fatty liver diseaseMetabolism60(3)404-413(2011) 3.Van Calcar, S.C., Baker, M.W., Williams, P., et al.Prevalence and mutation analysis of short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) detected on newborn screening in WisconsinMol. Genet. Metab.110(1-2)111-115(2013)