Reaction: MTR transfers CH3 group from 5-methyl-THF to cob(I)alamin
- in pathway: Cobalamin (Cbl) metabolism
Methionine synthase (MTR) mediates the continuous shuttling of cobalamin (Cbl) between two forms, cob(I)alamin and MeCbl. In this half reaction, the methyl group from 5 methyltetrahydrofolate (5-methyl-THF) is transferred to enzyme bound cob(I)alamin to form MeCbl (Hall et al. 2000). Defects in MTR cause methylcobalamin deficiency type G (cblG, methionine synthase deficiency; MIM:250940), an autosomal recessive inherited disease that causes mental retardation, macrocytic anemia, and homocystinuria (Leclerc et al. 1996).
Reaction - small molecule participants:
THF [cytosol]
5-methyl-THF [cytosol]
Reactome.org reaction link: R-HSA-3149539
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Reaction input - small molecules:
(6S)-5-methyltetrahydrofolate(2-)
Reaction output - small molecules:
(6S)-5,6,7,8-tetrahydrofolate(2-)
Reactome.org link: R-HSA-3149539