3-Hydroxyglutaric Acid

A biomarker for GCDH deficiency

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库存: 现货

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1mg

￥112.00

90.00

- +
5mg

￥600.00

480.00

- +
10mg

￥962.00

770.00

- +
25mg

￥1787.00

1430.00

- +

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货号: ajci67196
CAS: 638-18-6
别名: 3-羟基戊二酸
分子式: C5H8O5
分子量: 148.1
纯度: >98%
溶解度: DMF: 30 mg/ml,DMSO: 30 mg/ml,Ethanol: 30 mg/ml,PBS (pH 7.2): 10 mg/ml
储存: Store at -20°C
库存: 现货

Background

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.