Malonic Acid-d4

Malonic acid-d4 is intended for use as an internal standard for the quantification of malonic acid by GC- or LC-MS. Malonic acid is a dicarboxylic acid. At physiological pH, malonic acid is deprotonated to malonate, which competitively inhibits mitochondrial complex II, also known as succinate dehydrogenase, or is converted into malonyl-coenzyme A (malonyl-CoA, Item No. 16455) for use in fatty acid biosynthesis. Malonic and methylmalonic acid levels are elevated in combined malonic and methylmalonic aciduria, a disorder characterized by deficiency in malonyl-CoA decarboxylase (MCD) or acyl-CoA synthetase family member 3 (ACSF3).. SMILES: [2H]OC(C([2H])([2H])C(O[2H])=O)=O. InChi Code: InChI=1S/C3H4O4/c4-2(5)1-3(6)7/h1H2,(H,4,5)(H,6,7)/i1D2/hD2. InChi Key: OFOBLEOULBTSOW-BGOGGDMHSA-N