Methylmalonic Acid-d3

Methylmalonic acid-d3 is intended for use as an internal standard for the quantification of methylmalonic acid (Cay-14885) by GC- or LC-MS. Methylmalonic acid is a dicarboxylic acid and byproduct of propionate catabolism. It impairs mitochondrial respiration, increases glucose consumption, and downregulates the transcription of the mature neuronal markers neuron-specific enolase (ENO2) and synaptophysin (SYP) in neuronally differentiated SH-SY5Y cells. Intrastriatal administration of methylmalonic acid (100 mg/ml) induces neuronal loss in rats. Methylmalonic acid accumulates in the tissues and body fluids of patients with methylmalonic acidemia, an inborn error of metabolism characterized by a deficiency in the activity of L-methylmaolnyl-CoA mutase or its cofactor 5-deoxyadenosylcobalamin and leads to seizures, intellectual disabilities, psychomotor abnormalities, and coma.Formal Name: 2-methyl-d3-propanedioic acid. CAS Number: 42522-59-8. Synonyms: Isosuccinic Acid-d3, Methylmalonate-d3, MMA-d3. Molecular Formula: C4H3D3O4. Formula Weight: 121.1. Purity: >99% deuterated forms (d1-d3). Formulation: (Request formulation change), A solid. Solubility: DMSO: slightly, Methanol: slightly. SMILES: OC(C(C([2H])([2H])[2H])C(O)=O)=O. InChi Code: InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)/i1D3. InChi Key: ZIYVHBGGAOATLY-FIBGUPNXSA-N.