2-Methylbarnsteenzuur (standaard)

2-Methylsuccinic acid (Standard) is the analytical standard of 2-Methylsuccinic acid. This product is intended for research and analytical applications. 2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy.

498-21-5

Pyrotartaric acid (Standard)

12352211

Endogenous Metabolite; Reference Standards

Metabolic Enzyme/Protease; Others

Metabolic Disease

O=C(O)C(C)CC(O)=O

C5H8O4

132.12

[1]Grosso S, et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249 (10) :1446-50.|[2]Nowaczyk MJ, et al. Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul;47 (7) :836-9.

Room temperature

http://file.medchemexpress.com/batch_PDF/HY-W010381R/

Reference Standards