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DL-α-Hydroxyglutaric acid disodium salt
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
DL-α-Hydroxyglutaric acid disodium salt图片
CAS NO:40951-21-1
包装与价格:
包装价格(元)
10mg电议
25mg电议
100mg电议
250mg电议

产品介绍
alpha hydroxy acid
Cas No.40951-21-1
别名DL-Α-羟基戊二酸二钠
化学名sodium 2-hydroxypentanedioate
Canonical SMILESOC(C([O-])=O)CCC([O-])=O.[Na+].[Na+]
分子式C5H6Na2O5
分子量192.08
溶解度PBS (pH 7.2): 10 mg/ml
储存条件Store at -20℃
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping ConditionEvaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
产品描述

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 [1].

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 [2].

Clinical trial: Up to now, D-α-Hydroxyglutaric acid is still in the preclinical development stage.

References:
[1] Kranendijk M,Struys EA,van Schaftingen E,Gibson KM,Kanhai WA,van der Knaap MS,Amiel J,Buist NR,Das AM,de Klerk JB,Feigenbaum AS,Grange DK,Hofstede FC,Holme E,Kirk EP,Korman SH,Morava E,Morris A,Smeitink J,Sukhai RN,Vallance H,Jakobs C,Salomons GS.  IDH2 mutations in patients with D-2-hydroxyglutaric aciduria. Science.2010 Oct 15;330(6002):336.
[2] Lazovic J,Soto H,Piccioni D,Lou JR,Li S,Mirsadraei L,Yong W,Prins R,Liau LM,Ellingson BM,Cloughesy TF,Lai A,Pope WB.  Detection of 2-hydroxyglutaric acid in vivo by proton magnetic resonance spectroscopy in U87 glioma cells overexpressing isocitrate dehydrogenase-1 mutation. Neuro Oncol.2012 Dec;14(12):1465-72.