Mmachc
entrez
67096
canonical
uniprot
Q9CZD0
canonical
ensembl
ENSMUSG00000028690
resolver
ensp
ENSMUSP00000030453
resolver
enst
ENSMUST00000030453
resolver
genesymbol
Mmachc
resolver
uniprot
Q9D8S7
resolver
uniprot_entry
MMAC_MOUSE
resolver
uniprot_sec
Q9D8S7
resolver
amino_acid_sequence
MEPRVAELKQKIEDTLCPFGFEVYPFQVAWYNELLPPAFHLPFPGPTLAFLVLSTPAMFDRALKPFLKSCHFQTLRDPVDQCVSYHLRSVTEKFPEVHMEVIADYEVHPNRRPKILAQTAAHVAGAAYYYQRQDVDADPWGTQHIAGVCIHPRFGGWFAIRGVMLLPGIEVPNLPPRKPPDCVPTRAGRITLLEGFNFHWRDWTYRDAVTPEERYSEEQKIYFSTPPAQRLALLGLAQPSEHPSTTSELPLSLLTKPQNSRRARSWLSPSVSPPVSPGP
uniprot
proteins
ec_number
1.16.1.6
uniprot
proteins
ec_number
2.5.1.151
uniprot
proteins
function
FUNCTION: Cobalamin (vitamin B12) cytosolic chaperone that catalyzes the reductive decyanation of cyanocob(III)alamin (cyanocobalamin, CNCbl) to yield cob(II)alamin and cyanide, using FAD or FMN as cofactors and NADPH as cosubstrate. Cyanocobalamin constitutes the inactive form of vitamin B12 introduced from the diet, and is converted into the active cofactors methylcobalamin (MeCbl) involved in methionine biosynthesis, and 5'-deoxyadenosylcobalamin (AdoCbl) involved in the TCA cycle. Forms a complex with the lysosomal transporter ABCD4 and its chaperone LMBRD1, to transport cobalamin across the lysosomal membrane into the cytosol. The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine. Also acts as a glutathione transferase by catalyzing the dealkylation of the alkylcob(III)alamins MeCbl and AdoCbl, using the thiolate of glutathione for nucleophilic displacement to generate cob(I)alamin and the corresponding glutathione thioether. The conversion of incoming MeCbl or AdoCbl into a common intermediate cob(I)alamin is necessary to meet the cellular needs for both cofactors. Cysteine and homocysteine cannot substitute for glutathione in this reaction. {ECO:0000250|UniProtKB:Q9Y4U1}.
uniprot
proteins
mass_dalton
31648
uniprot
proteins
protein_family
MMACHC family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
21697092
uniprot
proteins
pubmed
24889031
uniprot
proteins
sequence_length
279
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Cytoplasm, cytosol {ECO:0000250|UniProtKB:Q9Y4U1}.
uniprot
proteins