Methylmalonyl-CoA mutase (MMUT)
Cofactors used
Clinical / pharmacological. Methylmalonic acidaemia; adenosylcobalamin (B12)-dependent
What it does, reaction by reaction
Propionyl-CoA to Succinyl-CoA (anaplerosis) Amino Acid & Nitrogen Metabolism · Mitochondrial matrix
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
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · fetched just now · sorted by publication date
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1
Targeting MMA-induced USP36 methylmalonylation to suppress macrophage polarization and tumor progression in clear-cell renal cell carcinoma.
Miao D, Shi J, Tan D, Zhao C, Lv Q, Lu F, Yang J, Yang H, Xiong Z, Zhang X. · 2025-12-15
cited 4× unreviewed -
2
Methylmalonate Overload Despite Glycemic Control Drives Diabetic Heart Damage.
Wang S, Yan M, Zhang Y, Tang F, Wang Y, Guo J, Chen Z, Liu X, Li Z, Lu R, Cui Y, Ma G, Wu P, Huang Y, Liu Y, … · 2026-07-29
unreviewed -
3
First Brazilian birth after Preimplantation Genetic Testing for Severe Methylmalonic Acidemia: preventing sudden infant death.
Fontes RB, Fontes CA, Sousa-Polezzi RC, Fontes RCFB, Ali TM, Cerveró A. · 2026-07-13
unreviewed -
4
Integrative proteogenomic analyses identify plasma proteins that impact the risk of ischemic stroke.
Belbasis L, von Ende A, Gajendragadkar P, Valdes-Marquez E, Murgia F, van Duijn C, Hopewell JC. · 2026-07-03
unreviewed -
5
Research Progress on Muttony Flavor Regulation and the Establishment of Its Digital Intelligent Evaluation System From a Global Perspective.
Wang Y, Liu W. · 2026-07-01
unreviewed -
6
Whole genome analysis of breed-specific copy number variations in dogs.
Wang S, Li Z, Bargmann W, Chen Z, Gruen J, Hoh J. · 2026-04-27
unreviewed -
7
Late-onset severe axonal polyneuropathy in mut0 methylmalonic acidemia after liver-kidney transplantation: a genotype-informed case report.
Griffo M, Magri F, Furlan F, Bernardi E, Menni F, Tagliaferri F, Testa S, Ronchi D, Petrillo S, Piemonte F, G… · 2026-06-11
unreviewed -
8
Multiplexed targeted mRNA profiling of alcohol-related liver disease reveals stage-specific dysregulation of signaling pathways.
Rasic D, Thiele M, Andersen P, Krag A, Detlefsen S. · 2026-06-09
open access unreviewed -
9
Association between serum methylmalonic acid and metabolic dysfunction-associated steatotic liver disease: NHANES-based study.
Zhang Z, Li J, Zeng J, Wang W, Liang X, Luo Y, Chen C, Luo Z, He J, Kong D, Ding Y. · 2026-06-01
open access unreviewed -
10
MCAK/Kif2C centromeric activity level tunes K-fiber turnover through distinct pathways.
Wagenbach M, Vicente JJ, Wordeman L. · 2026-05-26
open access unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.