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}
Technical Information
Formal Name
N-[3-(formylamino)-4-oxo-6-phenoxy-4H-1-benzopyran-7-yl]-methanesulfonamide
CAS Number
123663-49-0
Synonyms
IGU
T-614
Molecular Formula
C17H14N2O6S
Formula Weight
374.4
Purity
98%
Formulation
(Request formulation change)
A solid
Solubility
(Learn about Variance in Solubility)
DMF: 14 mg/ml
DMSO: 16 mg/ml
PBS (pH 7.2): 0.25 mg/ml
λmax
257, 264 nm
SMILES
O=CNC1=COC2=CC(NS(=O)(C)=O)=C(C=C2C1=O)OC3=CC=CC=C3
InChi Code
InChI=1S/C17H14N2O6S/c1-26(22,23)19-13-8-15-12(17(21)14(9-24-15)18-10-20)7-16(13)25-11-5-3-2-4-6-11/h2-10,19H,1H3,(H,18,20)
InChi Key
ANMATWQYLIFGOK-UHFFFAOYSA-N
Shipping & Storage Information
Storage -20°C
Shipping
Room Temperature in continental US; may vary elsewhere
Stability
Shelf life: 4 years