MTR transfers CH3 group from 5-methyl-THF to cob(I)alamin

Stable Identifier
R-HSA-3149539
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
8968737 Human methionine synthase: cDNA cloning and identification of mutations in patients of the cblG complementation group of folate/cobalamin disorders

Ross, M, Adjalla, CE, Eydoux, P, Gravel, RA, Rosenblatt, DS, Christensen, B, Leclerc, D, Campeau, E, Rozen, R, Goyette, P

Hum Mol Genet 1996
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Catalyst Activity

methionine synthase activity of MTRR:MTR:cob(I)alamin [cytosol]

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