A metabolite produced during GCDH deficiency and used as a biomarker of glutaric acidemia type 1

3-Hydroxyglutaric Acid – 5 mg

Brand:
Cayman
CAS:
638-18-6
Storage:
-20
UN-No:
Non-Hazardous - /

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.{13906} 3-Hydroxyglutaric acid is one of several metabolites produced when insufficient levels of GCDH are available.{16334,13906} 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.{16334,13906} 3-Hydroxyglutaric acid is used as a biomarker of GCDH deficiency.{16334,13906}  

 

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Description

A metabolite produced during GCDH deficiency and used as a biomarker of glutaric acidemia type 1

Formal name: 3-hydroxy-pentanedioic acid

Synonyms:  2,4-Dideoxypentaric Acid|3-HG|3-Hydroxyglutarate|3-Hydroxypentanedioic Acid|3-OH-GA|β-Hydroxyglutaric Acid

Molecular weight: 148.1

CAS: 638-18-6

Purity: ≥95%

Formulation: A crystalline solid

Product Type|Biochemicals|Lipids|Fatty Acids||Research Area|Cell Biology|Mitochondrial Biology||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Lipid Biochemistry||Research Area|Neuroscience|Neurodegenerative Disorders

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