3-<wbr/>Hydroxyglutaric Acid

3-Hydroxyglutaric Acid

CAT N°: 16334
Price:

From 55.00 46.75

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}

Territorial Availability: Available through Bertin Technologies only in France

  • Synonyms
    • 3-hydroxy-pentanedioic acid
  • Correlated keywords
    • 3-hydroxy glutaric 2,4-dideoxy pentaric 3HG glutarate pentanedioic 3-OHGA 3OHGA
  • Product Overview:
    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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