3-Hydroxyglutaric Acid (Synonyms: 2,4-Dideoxypentaric Acid, 3-HG, 3-Hydroxyglutarate, β-Hydroxyglutaric Acid, 3-Hydroxypentanedioic Acid, 3-OH-GA) |
| رقم الكتالوجGC41477 |
3-حمض الهيدروكسي جلوتاريك مشتق من حمض الجلوتاريك
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Cas No.: 638-18-6
Sample solution is provided at 25 µL, 10mM.
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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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