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L-2-Hydroxyglutaric acid disodium

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Chemical Structure| 63512-50-5 同义名 : S-2-羟基戊二酸 ;(S)-2-Hydroxyglutaric acid disodium;L-α-Hydroxyglutaric Acid (sodium salt);(S)-2-Hydroxypentanedioic Acid;L-2-Hydroxyglutaric Acid;L-2-Hydroxyglutarate;L-2HG;LGA;L-2-Hydroxyglutarate disodium
CAS号 : 63512-50-5
货号 : A754859
分子式 : C5H6Na2O5
纯度 : 95%
分子量 : 192.078
MDL号 : MFCD00021700
存储条件:

粉末 Inert atmosphere,2-8°C

液体 -20°C:3-6个月-80°C:12个月

溶解度 :

H2O: 85 mg/mL(442.53 mM),配合低频超声助溶

动物实验配方:
生物活性
描述 L-2-Hydroxyglutaric acid disodium is an epigenetic modifier and putative oncometabolite in renal cancer. L-2-Hydroxyglutaric acid disodium can inhibit histone demethylases and hence promote histone methylation. L-2-Hydroxyglutaric acid is potent at inhibiting 2-oxoglutarate (2-OG) dependent dioxygenases (2OGDs) including the Ten Eleven Translocation (TET) enzyme. 2HG can inhibit 2-oxoglutaratre (2-OG)-dependent dioxygenases that mediate epigenetic events, including DNA and histone demethylation[3]. L-2-Hydroxyglutaric acid (LGA) is the biochemical hallmark of patients affected by the neurometabolic disorder known as L-2-hydroxyglutaric aciduria (LHGA). tCK activity was significantly inhibited by LGA in the cerebellum, but not in cerebral cortex, cardiac muscle and skeletal muscle. Furthermore, CK activity from the mitochondrial fraction was inhibited by LGA, whereas that from the cytosolic fraction of cerebellum was not affected by the acid. The inhibitory effect of LGA on tCK was fully prevented by pre-incubation of the homogenates with reduced glutathione (GSH), suggesting that this inhibition is possibly mediated by oxidation of essential thiol groups of the enzyme[4]. L-2-HG was intracerebrally administered to rat pups at postnatal day 1 (P1) to induce a rise of L-2-HG (L-2-hydroxyglutaric acid) levels in the central nervous system (CNS). L-2-HG markedly induced the generation of reactive oxygen species (increase of 2',7'-dichloroflurescein-DCFH-oxidation), lipid peroxidation (increase of malondialdehyde concentrations), and protein oxidation (increase of carbonyl formation and decrease of sulfhydryl content), besides decreasing the antioxidant defenses (reduced glutathione-GSH) and sulfhydryl content in the cerebral cortex[5].
实验方案
1mg 5mg 10mg

1 mM

5 mM

10 mM

5.21mL

1.04mL

0.52mL

26.03mL

5.21mL

2.60mL

52.06mL

10.41mL

5.21mL

参考文献

[1]Shim EH, et al. L-2-Hydroxyglutarate: an epigenetic modifier and putative oncometabolite in renal cancer. Cancer Discov. 2014 Nov;4(11):1290-8.

[2]da Silva CG, et al. L-2-hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats. Int J Dev Neurosci. 2003 Jun;21(4):217-24.

[3]Shim EH, Livi CB, Rakheja D, Tan J, Benson D, Parekh V, Kho EY, Ghosh AP, Kirkman R, Velu S, Dutta S, Chenna B, Rea SL, Mishur RJ, Li Q, Johnson-Pais TL, Guo L, Bae S, Wei S, Block K, Sudarshan S. L-2-Hydroxyglutarate: an epigenetic modifier and putative oncometabolite in renal cancer. Cancer Discov. 2014 Nov;4(11):1290-8

[4]da Silva CG, Bueno AR, Schuck PF, Leipnitz G, Ribeiro CA, Wannmacher CM, Wyse AT, Wajner M. L-2-hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats. Int J Dev Neurosci. 2003 Jun;21(4):217-24

[5]Ribeiro RT, Zanatta Â, Amaral AU, Leipnitz G, de Oliveira FH, Seminotti B, Wajner M. Experimental Evidence that In Vivo Intracerebral Administration of L-2-Hydroxyglutaric Acid to Neonatal Rats Provokes Disruption of Redox Status and Histopathological Abnormalities in the Brain. Neurotox Res. 2018 Apr;33(3):681-692