Ethylmalonic acid (Standard)

Ethylmalonic acid (Standard) is the analytical standard of Ethylmalonic acid (HY-34740) . This product is intended for research and analytical applications. Ethylmalonic acid is a short-chain organic dicarboxylic acid. Ethylmalonic acid synergistically induces mitochondrial permeability transition (MP) with Ca2+, inhibits Mi-CK, and disrupts mitochondrial energy metabolism. Ethylmalonic acid can be used in the research of SCADD, EE and other genetic metabolic diseases characterized by EMA accumulation[1][2][3][4].

Product Specifications

CAS Number

[601-75-2]

UNSPSC

12352211

Target

Mitochondrial Metabolism; Reference Standards

Type

Reference Standards

Related Pathways

Metabolic Enzyme/Protease; Others

Field of Research

Metabolic Disease; Neurological Disease

Assay Protocol

https://www.medchemexpress.com/ethylmalonic-acid-standard.html

Purity

90.000000

Smiles

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

Molecular Formula

C5H8O4

Molecular Weight

132.11

References & Citations

[1]Gregersen N, et al. General (medium-chain) acyl-CoA dehydrogenase deficiency (non-ketotic dicarboxylic aciduria): quantitative urinary excretion pattern of 23 biologically significant organic acids in three cases. Clin Chim Acta. 1983 Aug 15;132(2):181-91.|[2]Yano S, et al. A new case of malonyl coenzyme A decarboxylase deficiency presenting with cardiomyopathy. Eur J Pediatr. 1997 May;156(5):382-3.