MUT isomerises L-MM-CoA to SUCC-CoA
L-MM-CoA → SUCC-CoA
- Reactome
- R-HSA-71010
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:22889
Inputs
- L-MM-CoACHEBI:57326, C25H35N7O19P3S, mitochondrial matrix
Outputs
- SUCC-CoACHEBI:57292, C25H35N7O19P3S, mitochondrial matrix
Catalysed by
- methylmalonyl-CoA mutase activity of 2xMMAA:2xMUT:AdoCbl [mitochondrial matrix]EC 5.4.99.2
Literature
- Cloning of full-length methylmalonyl-CoA mutase from a cDNA library using the polymerase chain reaction Genomics, 1989
- Spectrum of mutations in mut methylmalonic acidemia and identification of a common Hispanic mutation and haplotype Hum. Mutat., 2006
- Structures of the human GTPase MMAA and vitamin B12-dependent methylmalonyl-CoA mutase and insight into their complex formation J. Biol. Chem., 2010
- Protection and reactivation of human methylmalonyl-CoA mutase by MMAA protein Biochem. Biophys. Res. Commun., 2011
- Assembly and protection of the radical enzyme, methylmalonyl-CoA mutase, by its chaperone Biochemistry, 2006
- Identification of the gene responsible for the cblA complementation group of vitamin B12-responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements Proc. Natl. Acad. Sci. U.S.A., 2002