Methylmalonate - CAS 516-05-2
Category: Inhibitor
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Molecular Formula:
Molecular Weight:
Methylmalonate, a dicarboxylic acid that can be derived from methylmalonyl-coenzyme A (methylmalonyl-CoA), binds with coenzyme A to form methylmalonyl-CoA. It is a reversible succinate dehydrogenase inhibitor used to generate Huntington's disease models and induces apoptotic cell death in striatal neurons.
2-Methylpropanedioic acid;
Isosuccinic Acid; Methylmalonic Acid; MMA; NSC 25201
Store in a cool and dry place (or refer to the Certificate of Analysis).
Canonical SMILES:
1.Carglumic acid enhances rapid ammonia detoxification in classical organic acidurias with a favourable risk-benefit profile: a retrospective observational study.
Valayannopoulos V1, Baruteau J2, Delgado MB3, Cano A4, Couce ML5, Del Toro M6, Donati MA7, Garcia-Cazorla A8, Gil-Ortega D9, Gomez-de Quero P10, Guffon N11, Hofstede FC12, Kalkan-Ucar S13, Coker M13, Lama-More R14, Martinez-Pardo Casanova M15, Molina A16, Pichard S2, Papadia F17, Rosello P16, Plisson C18, Le Mouhaer J18, Chakrapani A2. Orphanet J Rare Dis. 2016 Mar 31;11(1):32. doi: 10.1186/s13023-016-0406-2.
BACKGROUND: Isovaleric aciduria (IVA), propionic aciduria (PA) and methylmalonic aciduria (MMA) are inherited organic acidurias (OAs) in which impaired organic acid metabolism induces hyperammonaemia arising partly from secondary deficiency of N-acetylglutamate (NAG) synthase. Rapid reduction in plasma ammonia is required to prevent neurological complications. This retrospective, multicentre, open-label, uncontrolled, phase IIIb study evaluated the efficacy and safety of carglumic acid, a synthetic structural analogue of NAG, for treating hyperammonaemia during OA decompensation.
2.Low Serum Vitamin B-12 Concentrations Are Prevalent in a Cohort of Pregnant Canadian Women.
Visentin CE1, Masih SP1, Plumptre L1, Schroder TH2, Sohn KJ3, Ly A4, Lausman AY5, Berger H5, Croxford R6, Lamers Y2, Kim YI7, O'Connor DL8. J Nutr. 2016 Apr 13. pii: jn226845. [Epub ahead of print]
BACKGROUND: Among Canadian women of reproductive age, 5% and 20% have serum vitamin B-12 concentrations indicative of deficiency (<148 pmol/L) and marginal status (148-220 pmol/L), respectively. Given the association between suboptimal vitamin B-12 and adverse pregnancy outcomes, an understanding of vitamin B-12 status during pregnancy, and factors that influence it, is required.
3.Methylmalonic acid and neutrophil morphometric index in laboratory diagnosis of cobalamin deficiency without macrocytosis.
Totoskovic D1, Dopsaj V1,2, Martinovic J3. Int J Lab Hematol. 2016 Mar 19. doi: 10.1111/ijlh.12479. [Epub ahead of print]
INTRODUCTION: This study aimed to investigate the utility of methylmalonic acid (MMA) and neutrophil Cell Population Data, available on the Beckman Coulter LH750 Analyser (Miami, FL, USA), in laboratory assessment of cobalamin status in patients at risk of cobalamin deficiency, without macrocytosis and inflammation.
4.Neurologic, psychiatric, and other medical manifestations of nitrous oxide abuse: A systematic review of the case literature.
Garakani A1,2,3, Jaffe RJ1, Savla D4, Welch AK1, Protin CA5, Bryson EO1,6, McDowell DM. Am J Addict. 2016 Apr 1. doi: 10.1111/ajad.12372. [Epub ahead of print]
BACKGROUND/OBJECTIVES: Nitrous oxide (N2 O) is known to have abuse potential, although debate regarding the toxic effects of such abuse continues. Our objective was to review the case literature and present the neurologic, psychiatric and medical consequences of N2 O abuse.
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CAS 516-05-2 Methylmalonate

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