IC50: N/A D-α-Hydroxyglutaric acid, an α-hydroxy acid formed from the hydrolysis of (R)-5-oxo-2-tetrahydrofurancarboxylic acid, is over-produced in the human neurometabolic disease D-α-hydroxyglutaric aciduria. In vitro: In humans the enzyme hydroxyacid-oxoacid transhydrogenase catalyzed the formation of D-α-hydroxyglutaric acid, while D-α-hydroxyglutaric acid is catalyzed by 2-hydroxyglutarate synthase in bacteria. Recent study discovered heterozygous somatic mutations in the genes encoding isocitrate dehydrogenase-1 and -2 (IDH1 and IDH2) and these mutations could disable the enzymes' ability to convert 2-ketoglutarate to D-α-hydroxyglutarate. The authors also found heterozygous germline mutations in IDH2 that alter enzyme residue in patients with D-2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by supraphysiological levels of D-α-Hydroxyglutaric acid . In vivo: Previous animal study demonstrated that the overexpression of the isocitrate dehydrogenase-1 (IDH1) mutation resulted in detectable levels of D-α-Hydroxyglutaric acid in a mouse glioma model that could be measured in vivo and noninvasively by MRS. Results showed that this mouse model system was of potential application in establishing the relationships among D-α-Hydroxyglutaric acid levels and treatment effect, outcomes, as well as other related and necessary variables to validate D-α-Hydroxyglutaric acid as a clinically important biomarker for glioma . Clinical trial: Up to now, D-α-Hydroxyglutaric acid is still in the preclinical development stage.

Product information

CAS Number: 40951-21-1

Molecular Weight: 192.08

Formula: C5H6Na2O5

Chemical Name: disodium 2-hydroxypentanedioate

Smiles: [Na+].[Na+].[O-]C(=O)CCC(O)C([O-])=O

InChiKey: DZHFTEDSQFPDPP-UHFFFAOYSA-L

InChi: InChI=1S/C5H8O5.2Na/c6-3(5(9)10)1-2-4(7)8;;/h3,6H,1-2H2,(H,7,8)(H,9,10);;/q;2*+1/p-2

Technical Data

Appearance: Solid Power

Purity: ≥98% (or refer to the Certificate of Analysis)

Shipping Condition: Shipped under ambient temperature as non-hazardous chemical or refer to Certificate of Analysis

Storage Condition: Dry, dark and -20 ^oC for 1 year or refer to the Certificate of Analysis.

Shelf Life: ≥12 months if stored properly.

Stock Solution Storage: 0 - 4 ^oC for 1 month or refer to the Certificate of Analysis.

Drug Formulation: To be determined

HS Tariff Code: 382200

Products are for research use only. Not for human use.