Skip to content
MS
Sign in
unreviewed

Methylmalonyl-CoA mutase (MMUT)

1 reactions · 1 pathways

Cofactors used

Cobalamin (B12) · B12 (Cobalamin)

Clinical / pharmacological. Methylmalonic acidaemia; adenosylcobalamin (B12)-dependent

What it does, reaction by reaction

1 reactions

Propionyl-CoA to Succinyl-CoA (anaplerosis) Amino Acid & Nitrogen Metabolism · Mitochondrial matrix

step 3 Irreversible/directional

L-Methylmalonyl-CoA -> succinyl-CoA

Converts L-Methylmalonyl-CoA into Succinyl-CoA

› Notes

Requires adenosylcobalamin. B12 deficiency raises serum methylmalonic acid, which distinguishes it from folate deficiency; inherited defects cause methylmalonic acidaemia.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

Refresh

Europe PMC · fetched just now · sorted by publication date

  1. 1
    Essential Oral Single Nutritional Therapy Products for Inherited Metabolic Diseases: Evidence and Consensus Assessment Using a Modified Delphi Method.

    Stolwijk NN, Penninx BMF, Bosch AM, Langeveld M, Salomons GS, Hollak CEM, Essential Nutritional Therapy Produ… · 2026-11-01

    open access unreviewed
  2. 2
    Mitochondria-to-plasma-membrane H<sub>2</sub>O<sub>2</sub> signaling induced by branched-chain keto acid oxidation essentially stimulates insulin secretion in pancreatic β-cells.

    Klöppel E, Engstová H, Duspiva P, Tauber J, Mozheitova O, Jabůrek M, Ježek P. · 2026-07-16

    open access unreviewed
  3. 3
    Exploration of Metabolic Alterations in Methylmalonic Acidemia across Distinct Genetic Backgrounds.

    Zhi HJ, Ding Y, Deng YX, Chen T, Cao Y, Chang SY, Zhan X, Xu F, Hao LL, Liang LL, Qiu WJ, Zhang HW, Gu XF, Ha… · 2026-09-07

    open access unreviewed
  4. 4
    Disturbances of Mitochondrial Functions and Oxidative Stress Induction in Methylmalonic and Propionic Acidemias: A Critical Role for Metabolite Accumulation.

    Amaral AU, Wajner M. · 2026-09-20

    open access unreviewed
  5. 5
    Sarcopenia in Pediatric Intoxication Type Inborn Errors of Metabolism: A Frequent and Underrecognized Condition.

    Veraldi S, Basso MS, Soglia G, Cirelli R, Spagnoletti G, Bernabei SM, Esposito A, Rizzo C, Cairoli S, Martine… · 2026-09-01

    cited 1× unreviewed
  6. 6
    Mitochondrial depolarization stabilizes the vitamin B<sub>12</sub> chaperone MMADHC in the cytosol to increase MTR activity.

    Rath SP, Li Z, Guha A, Dong F, Banerjee R, Mootha VK. · 2026-09-08

    cited 1× open access unreviewed
  7. 7
    Shotgun proteomic analysis of quail liver under feed deprivation and low-energy diet conditions.

    Khalifeh DM, Gulyás G, Knop R, Reda GK, Kalló G, Szabó C, Csösz É, Czeglédi L. · 2026-08-11

    open access unreviewed
  8. 8
    Irritable Bowel Disease: Think Malonic and Methylmalonic Aciduria (CMAMMA)!

    Alatibi K, Hug MJ, Tucci S. · 2026-08-23

    open access unreviewed
  9. 9
    Liver-directed lentiviral gene therapy confers durable hepatic and systemic amelioration of methylmalonic acidemia in mice.

    Barbon E, Simoni C, Argabright A, Boettiger M, Negri C, Manta E, Raimondi A, Vezzoli E, Canepari C, Biffi M, … · 2026-05-25

    unreviewed
  10. 10
    Early Cardiomyopathy in Prediabetic NDPK-B-Deficient Mice Is Associated with Remodeling of the Mitochondrial O-GlcNAc Proteome.

    Karim N, Qin M, Eshwaran R, Shao F, Lomada S, Keles M, Wang Y, Trogisch FA, Schlattner U, Heineke J, Wieland … · 2026-08-22

    open access unreviewed

External claims. These come from an index outside this database and are not checked against it. Treat them as leads.