Formulation: A solid
Formal Name: 2-methyl-d3-propanedioic acid
Purity: ≥99% deuterated forms (d1-d3)
Formula Markup: C4H3D3O4
Formula Weight: 121,1
Shelf life (days): 1460
CAS Number: 42522-59-8
Notes: Methylmalonic acid-d3 is intended for use as an internal standard for the quantification of methylmalonic acid (Item No. 14885) by GC- or LC-MS. Methylmalonic acid is a dicarboxylic acid and byproduct of propionate catabolism.{64351} 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.{64352} 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.{64351}
English
Deutsch
