SMART: Pfam domain MMACHC

The domain within your query sequence starts at position 20 and ends at position 234; the E-value for the MMACHC domain shown below is 9.5e-102.

GFEVYPFQVAWYNELLPPAFHLPFPGPTLAFLVLSTPAMFDRALKPFLKSCHFQTLRDPV
DQCVSYHLRSVTEKFPEVHMEVIADYEVHPNRRPKILAQTAAHVAGAAYYYQRQDVDADP
WGTQHIAGVCIHPRFGGWFAIRGVMLLPGIEVPNLPPRKPPDCVPTRAGRITLLEGFNFH
WRDWTYRDAVTPEERYSEEQKIYFSTPPAQRLALL

MMACHC

PFAM accession number:	PF16690
Interpro abstract (IPR032037):	MMACHC, also known as Cyanocobalamin reductase / alkylcobalamin dealkylase, is involved in the intracellular processing of vitamin B12 by catalyzing two reactions: the reductive decyanation of cyanocobalamin in the presence of a flavoprotein oxidoreductase and the dealkylation of alkylcobalamins through the nucleophilic displacement of the alkyl group by glutathione [ (PUBMED:18779575) (PUBMED:19801555) ]. Mutations in the MMACHC gene have been linked to a disease known as Methylmalonic aciduria and homocystinuria, cblC type (OMIM 277400), the most common inborn error of vitamin B(12) (cobalamin) metabolism. The C-terminal region of this protein folds similarly to TonB, a bacterial protein involved in energy transduction for cobalamin uptake [ (PUBMED:16311595) ]. The structure of MMACHC reveals it to be the most divergent member of the NADPH-dependent flavin reductase family that can use FMN or FAD to catalyze reductive decyanation; it is also the first enzyme with glutathione transferase (GST) activity that is unrelated to the GST superfamily in structure and sequence [ (PUBMED:21697092) ].

PFAM accession number:

PF16690

Interpro abstract (IPR032037):

MMACHC, also known as Cyanocobalamin reductase / alkylcobalamin dealkylase, is involved in the intracellular processing of vitamin B12 by catalyzing two reactions: the reductive decyanation of cyanocobalamin in the presence of a flavoprotein oxidoreductase and the dealkylation of alkylcobalamins through the nucleophilic displacement of the alkyl group by glutathione [ (PUBMED:18779575) (PUBMED:19801555) ]. Mutations in the MMACHC gene have been linked to a disease known as Methylmalonic aciduria and homocystinuria, cblC type (OMIM 277400), the most common inborn error of vitamin B(12) (cobalamin) metabolism. The C-terminal region of this protein folds similarly to TonB, a bacterial protein involved in energy transduction for cobalamin uptake [ (PUBMED:16311595) ].

The structure of MMACHC reveals it to be the most divergent member of the NADPH-dependent flavin reductase family that can use FMN or FAD to catalyze reductive decyanation; it is also the first enzyme with glutathione transferase (GST) activity that is unrelated to the GST superfamily in structure and sequence [ (PUBMED:21697092) ].

This is a PFAM domain. For full annotation and more information, please see the PFAM entry MMACHC