MTR transfers CH3 group from MTHF to cob(I)alamin

Stable Identifier
R-HSA-3149539
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
Summation

Methionine synthase (MTR) catalyses the transfer of a methyl group from 5-methyltetrahydrofolate (MTHF) to homocysteine (HCYS) to then form methionine (L-Met). In the first step, MTR mediates the transfer of a methyl group from 5-methyltetrahydrofolate (MTHF) to cob(I)alamin (B12s, bound to the enzyme MTR) to form the cofactor methylcobalamin (MeCbl), the form that activates MTR (Leclerc et al. 1996). 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).

Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
methionine synthase activity of MTRR:MTR(cob(I)alamin) [cytosol]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed