An α-hydroxy acid; metabolized to α-ketoglutarate by L-2-hydroxyglutarate dehydrogenase; levels are increased in 2-hydroxyglutaric aciduria; inhibits α-ketoglutarate-dependent oxygenases

L-α-Hydroxyglutaric Acid – 10 mg

Brand:
Cayman
CAS:
13095-48-2
Storage:
-20
UN-No:
De Minimis - 1170 / 3

L-α-Hydroxyglutaric acid (L-2-HG) is an α-hydroxy acid. It is metabolized to 2-oxoglutarate (α-ketoglutarate) by L-2-hydroxyglutarate dehydrogenase, and mutations in this enzyme lead to 2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by increased L-2-HG levels.{26770,26771} L-2-HG is structurally similar to α-ketoglutarate and competitively inhibits α-ketoglutarate-dependent dioxygenases, including several involved in histone lysine and DNA demethylation.{26769,26774}  

 

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Description

An α-hydroxy acid; metabolized to α-ketoglutarate by L-2-hydroxyglutarate dehydrogenase; levels are increased in 2-hydroxyglutaric aciduria; inhibits α-ketoglutarate-dependent oxygenases, including several involved in histone lysine and DNA demethylation

Formal name: 2S-hydroxy-pentanedioic acid

Synonyms:  2(S)-HG|2(S)-Hydroxyglutaric Acid|L-2-HG|L-2-Hydroxyglutaric Acid

Molecular weight: 148.1

CAS: 13095-48-2

Purity: ≥95%

Formulation: A solution in ethanol

Product Type|Biochemicals|Small Molecule Inhibitors|Demethylases||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Epigenetics, Transcription, & Translation|Erasers|DNA/RNA Demethylation||Research Area|Epigenetics, Transcription, & Translation|Erasers|Histone Demethylation||Research Area|Neuroscience

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