D-?-Hydroxyglutaric Acid (sodium

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D-?-Hydroxyglutaric Acid (sodium salt)

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Cat No: 11605

Biochemicals - More Biochemicals

D-α-Hydroxyglutaric Acid (D-2-HG) is an α-hydroxy acid that is over-produced in the human neurometabolic disease D-2-hydroxyglutaric aciduria (D-2-HGA).{26892} It is normally synthesized from 2-ketoglutarate (2-KG) by hydroxyacid-oxoacid transhydrogen...

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Territorial Availability: Available through Bertin Technologies only in France

Synonyms:

2R-hydroxy-pentanedioic acid, disodium salt

Correlated keywords:

neurosciences metabolisms biochemicals 13095-47-1 ?-hydroxy humans neurometabolic diseases D-2-hydroxyglutaric aciduria D-2-HGA D2HGA 2-ketoglutarate 2-KG 2KG hydroxyacid-oxoacid transhydrogenase HOT defects types I II mutations D-2-hydroxyglutarate dehydrogenase D-2-HGDH D2HGDH gain-of-function gains of functions isocitrates dehydrogenases 2 supplements bacteria oxalates propionyl-coenzyme A CoA propionyls conenzymes synthetases oxalate-coenzyme

Product Overview:
D-α-Hydroxyglutaric Acid (D-2-HG) is an α-hydroxy acid that is over-produced in the human neurometabolic disease D-2-hydroxyglutaric aciduria (D-2-HGA).{26892} It is normally synthesized from 2-ketoglutarate (2-KG) by hydroxyacid-oxoacid transhydrogenase (HOT), although defects in HOT are not known to be associated with D-2-HGA.{26892} Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG.{26892} Type II D-2-HGA results from gain-of-function mutations in isocitrate dehydrogenase 2, causing it to supplement HOT in converting 2-KG to D-2-HG.{26891,26772} In bacteria, this α-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an α-hydroxyglutaric acid synthetase.{26893}

Size	10 mg
Shipping	dry ice
CAS Number	103404-90-6
Molecular Formula	C₅H₆O₅ • 2Na
SMILES	[O-]C(CC[C@@H](O)C([O-])=O)=O.[Na+].[Na+]
Molecular Weight	192,1
Formulation	A crystalline solid
Purity	≥95%
Custom Code	2915.60
UNSPSC code	12352100

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