3-Hydroxyglutaric Acid
货号:
16334-1mg 基本售价:
406.0 元 规格:
1 mg
产品信息
概述货号 | 16334-1mg |
描述 | Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial matrix protein that catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide in the catabolic pathways of lysine, hydroxylysine, and tryptophan metabolism.1 3-Hydroxyglutaric acid is one of several metabolites produced when insufficient levels of GCDH are available.2,1 Urinary levels of 3-hydroxyglutaric acid are elevated during glutaric acidemia type 1, an autosomal recessive GCDH deficiency disorder that can lead to neurodegeneration if left untreated.2,1 3-Hydroxyglutaric acid is used as a biomarker of GCDH deficiency.2,1 |
别名 | 2,4-Dideoxypentaric Acid;3-HG;3-Hydroxyglutarate;β-Hydroxyglutaric Acid;3-Hydroxypentanedioic Acid;3-OH-GA; |
性能供应商 | Cayman |
应用文献 |
1.Sauer, S.W.,Okun, J.G.,Schwab, M.A., et al. Bioenergetics in glutaryl-coenzyme a dehydrogenase deficiency. A role for glutaryl-coenzyme A. The Journal of Biological Chemisty 290(23), 21830-21836 (2005). 2.Fang, N. and Casida, J.E. Anticancer action of cubé insecticide: Correlation for rotenoid constituents between inhibition of NADH: Ubiquinone oxidoreductase and induced ornithine decarboxylase activities. Proceedings of the National Academy of Sciences of the United States of America 95, 3380-3384 (1998).
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运输条件 | Room temperature in continental US; may vary elsewhere |
存放说明 | -20 |
纯度 | ≥95% |
计算分子量 | 148.1 |
分子式 | C5H8O5 |
CAS号 | 638-18-6 |
稳定性 | ≥ 2 years |
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