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Proteins/UNIPROT:Q9UBK8
Key enzyme in methionine and folate homeostasis responsible for the reactivation of methionine synthase (MTR/MS) activity by catalyzing the reductive methylation of MTR-bound cob(II)alamin. Cobalamin (vitamin B12) forms a complex with MTR to serve as an intermediary in methyl transfer reactions that cycles between MTR-bound methylcob(III)alamin and MTR bound-cob(I)alamin forms, and occasional oxidative escape of the cob(I)alamin intermediate during the catalytic cycle leads to the inactive cob(II)alamin species (Probable). The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR and MTR which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine. Also necessary for the utilization of methyl groups from the folate cycle, thereby affecting transgenerational epigenetic inheritance (By similarity). Also acts as a molecular chaperone for methionine synthase by stabilizing apoMTR and incorporating methylcob(III)alamin into apoMTR to form the holoenzyme. Also serves as an aquacob(III)alamin reductase by reducing aquacob(III)alamin to cob(II)alamin; this reduction leads to stimulation of the conversion of apoMTR and aquacob(III)alamin to MTR holoenzyme..
Function from UniProtKB (Q9UBK8), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
1 row
87 statements
87 statements · 7 reference databases · Subject: MTRR · Sources: Bgee, Hetionet, Human Protein Atlas, Reactome, Rhea, UniProt, WikiPathways
3 reactions
2MAM00188c+NADPH(4-)+2S-adenosyl-L-methionine2MAM00189c+proton+NADP(3-)+2CHEBI:16680
Catalysed by MTRR
Reaction pageReversibleBalance undeterminableEC 1.16.1.8cytosol
Human-GEM MAR09550CC BY 4.0Reference data
2cob(II)alamin+A+2water+2
3 names
Resolves to: MTRR
Catalysed by MTRR
Reaction pageRight to leftBalanced
Rhea RHEA:20752CC BY 4.0Reference data
2methylcob(III)alamin-[methionine synthase]+2S-adenosyl-L-homocysteine+NADP(3-)+hydron2cob(II)alamin-[methionine synthase]+2S-adenosyl-L-methionine+NADPH(4-)
Catalysed by MTRR
Reaction pageRight to leftBalance undeterminable
Rhea RHEA:23908CC BY 4.0Reference data