上海一研生物科技有限公司

产品属性：

Cas No.: 638-18-6

别名: N/A

化学名: N/A

分子式: C5H8O5

分子量: 148.1

溶解度: DMF: 30 mg/ml,DMSO: 30 mg/ml,Ethanol: 30 mg/ml,PBS (pH 7.2): 10 mg/ml

储存条件: Store at -20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.

Shipping ConditionEvaluation sample solution : ship with blue ice

All other available size: ship with RT , or blue ice upon request

产品描述：

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. 3-Hydroxyglutaric acid is one of several metabolites produced when insufficient levels of GCDH are available. 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. 3-Hydroxyglutaric acid is used as a biomarker of GCDH deficiency.
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