Mmachc

Functionⁱ

Catalyzes the reductive dealkylation of cyanocobalamin to cob(II)alamin, using FAD or FMN as cofactor and NADPH as cosubstrate. Can also catalyze the glutathione-dependent reductive demethylation of methylcobalamin, and, with much lower efficiency, the glutathione-dependent reductive demethylation of adenosylcobalamin. Under anaerobic conditions cob(I)alamin is the first product; it is highly reactive and is converted to aquocob(II)alamin in the presence of oxygen. Binds cyanocobalamin, adenosylcobalamin, methylcobalamin and other, related vitamin B12 derivatives.By similarity

Cofactorⁱ

FADBy similarity, FMNBy similarityNote: Can utilize both FAD and FMN.By similarity

Pathwayⁱ: adenosylcobalamin biosynthesis

This protein is involved in the pathway adenosylcobalamin biosynthesis, which is part of Cofactor biosynthesis.By similarity
View all proteins of this organism that are known to be involved in the pathway adenosylcobalamin biosynthesis and in Cofactor biosynthesis.

Sites

Feature key	Position(s)	DescriptionActions	Length
Binding siteⁱ	104	SubstrateBy similarity	1
Binding siteⁱ	149	Substrate; via amide nitrogenBy similarity	1
Binding siteⁱ	160	Substrate; via amide nitrogen and carbonyl oxygenBy similarity	1

GO - Molecular functionⁱ

cobalamin binding Source: UniProtKB-KW
cyanocobalamin reductase (cyanide-eliminating) activity Source: UniProtKB
demethylase activity Source: UniProtKB
FAD binding Source: UniProtKB
glutathione binding Source: UniProtKB
oxidoreductase activity Source: UniProtKB
protein homodimerization activity Source: MGI

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

cobalamin biosynthetic process Source: UniProtKB
cobalamin metabolic process Source: UniProtKB
demethylation Source: UniProtKB
glutathione metabolic process Source: UniProtKB
oxidation-reduction process Source: UniProtKB

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Oxidoreductase
Ligand	Cobalamin, Cobalt, FAD, Flavoprotein, FMN, NADP

Enzyme and pathway databases

Reactomeⁱ	R-MMU-196741 Cobalamin (Cbl, vitamin B12) transport and metabolism
UniPathwayⁱ	UPA00148

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Methylmalonic aciduria and homocystinuria type C protein homolog (EC:1.16.1.-By similarity) Alternative name(s): CblCBy similarity Cyanocobalamin reductase (cyanide-eliminating)
Gene namesⁱ	Name:Mmachc
Organismⁱ	Mus musculus (Mouse)
Taxonomic identifierⁱ	10090 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus
Proteomesⁱ	UP000000589 Componentⁱ: Chromosome 4

Organism-specific databases

MGIⁱ	MGI:1914346 Mmachc

Keywords - Cellular componentⁱ

Cytoplasm

Pathology & Biotechⁱ

Disruption phenotypeⁱ

Complete embryonic lethality. All die before 3.5 dpc.1 Publication

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000076259	1 – 279	Methylmalonic aciduria and homocystinuria type C protein homologAdd BLAST		279

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Modified residueⁱ	247	PhosphoserineBy similarity	1
Modified residueⁱ	272	PhosphoserineBy similarity	1
Modified residueⁱ	276	PhosphoserineBy similarity	1

Keywords - PTMⁱ

Phosphoprotein

Proteomic databases

PaxDbⁱ	Q9CZD0
PRIDEⁱ	Q9CZD0

PTM databases

iPTMnetⁱ	Q9CZD0
PhosphoSitePlusⁱ	Q9CZD0

Expressionⁱ

Tissue specificityⁱ

Detected in liver and kidney (at protein level) (PubMed:21697092). Detected in embryos (PubMed:24889031).2 Publications

Gene expression databases

Bgeeⁱ	ENSMUSG00000028690 Expressed in 266 organ(s), highest expression level in gastrocnemius
Genevisibleⁱ	Q9CZD0 MM

Interactionⁱ

Subunit structureⁱ

Monomer in the absence of bound substrate. Homodimer; dimerization is triggered by binding to FMN or adenosylcobalamin. Heterodimer with MMADHC.By similarity

GO - Molecular functionⁱ

protein homodimerization activity Source: MGI

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

STRINGⁱ	10090.ENSMUSP00000030453

STRINGⁱ

10090.ENSMUSP00000030453

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q9CZD0
SMRⁱ	Q9CZD0
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Region

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Regionⁱ	115 – 118	Substrate bindingBy similarity		4
Regionⁱ	129 – 131	Substrate bindingBy similarity		3

Sequence similaritiesⁱ

Belongs to the MMACHC family.Curated

Phylogenomic databases

eggNOGⁱ	ENOG410IKBC Eukaryota ENOG410XTCP LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000003464
HOGENOMⁱ	HOG000231413
HOVERGENⁱ	HBG080267
InParanoidⁱ	Q9CZD0
KEGG Orthology (KO) More...KOⁱ	K14618
OMAⁱ	MEPQVAE
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0GLW
PhylomeDBⁱ	Q9CZD0
TreeFamⁱ	TF332476

Family and domain databases

Conserved Domains Database More...CDDⁱ	cd12959 MMACHC-like, 1 hit
InterProⁱ	View protein in InterPro IPR032037 MMACHC
PANTHERⁱ	PTHR31457 PTHR31457, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF16690 MMACHC, 1 hit

Sequenceⁱ

Sequence statusⁱ: Complete.

Q9CZD0-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MEPRVAELKQ KIEDTLCPFG FEVYPFQVAW YNELLPPAFH LPFPGPTLAF 
        60         70         80         90        100
LVLSTPAMFD RALKPFLKSC HFQTLRDPVD QCVSYHLRSV TEKFPEVHME 
       110        120        130        140        150
VIADYEVHPN RRPKILAQTA AHVAGAAYYY QRQDVDADPW GTQHIAGVCI 
       160        170        180        190        200
HPRFGGWFAI RGVMLLPGIE VPNLPPRKPP DCVPTRAGRI TLLEGFNFHW 
       210        220        230        240        250
RDWTYRDAVT PEERYSEEQK IYFSTPPAQR LALLGLAQPS EHPSTTSELP 
       260        270 
LSLLTKPQNS RRARSWLSPS VSPPVSPGP

Length:279

Mass (Da):31,648

Last modified:January 10, 2006 - v2

Checksum:ⁱ178FAEB388691B09

GO

Sequence cautionⁱ

The sequence AAH54756 differs from that shown. Reason: Erroneous initiation.Curated

The sequence BAB25214 differs from that shown. Reason: Frameshift at position 13.Curated

The sequence BAC39135 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature key	Position(s)	DescriptionActions	Length
Sequence conflictⁱ	14	D → G in BAB28451 (PubMed:16141072).Curated	1
Sequence conflictⁱ	239	P → H in BAB28451 (PubMed:16141072).Curated	1
Sequence conflictⁱ	256	K → T in BAB28451 (PubMed:16141072).Curated	1
Sequence conflictⁱ	260	S → Y in BAB28451 (PubMed:16141072).Curated	1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AK007725 mRNA Translation: BAB25214.1 Frameshift. AK012761 mRNA Translation: BAB28451.1 AK084194 mRNA Translation: BAC39135.1 Different initiation. BC054756 mRNA Translation: AAH54756.1 Different initiation.
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS51278.1
NCBI Reference Sequences More...RefSeqⁱ	NP_080238.2, NM_025962.3
UniGeneⁱ	Mm.252785

Genome annotation databases

Ensemblⁱ	ENSMUST00000030453; ENSMUSP00000030453; ENSMUSG00000028690
GeneIDⁱ	67096
KEGGⁱ	mmu:67096
UCSC genome browser More...UCSCⁱ	uc008uhe.1 mouse

Similar proteinsⁱ

90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q9CZD0	Metabolism of cobalamin-associated C		RAT		279	UniRef90_Q9CZD0
methylmalonic aciduria and homocystinuria type C protein		MESAU		279
Methylmalonic aciduria and homocystinuria type C-like		CRIGR		279
Uncharacterized protein		NEOLE		276
UPI00042AAD1F		PERMB		279
+7

Protein	Similar proteins		Species	Score	Length	Source
Q9CZD0	Methylmalonic aciduria and homocystinuria type C protein homolog		CHICK		238	UniRef50_Q9CZD0
methylmalonic aciduria and homocystinuria type C protein		MESAU		279
Methylmalonic aciduria and homocystinuria type C-like		CRIGR		279
Metabolism of cobalamin-associated C		RAT		279
Uncharacterized protein		NEOLE		276
+100

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AK007725 mRNA Translation: BAB25214.1 Frameshift. AK012761 mRNA Translation: BAB28451.1 AK084194 mRNA Translation: BAC39135.1 Different initiation. BC054756 mRNA Translation: AAH54756.1 Different initiation.
CCDSⁱ	CCDS51278.1
RefSeqⁱ	NP_080238.2, NM_025962.3
UniGeneⁱ	Mm.252785

3D structure databases

ProteinModelPortalⁱ	Q9CZD0
SMRⁱ	Q9CZD0
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

STRINGⁱ	10090.ENSMUSP00000030453

STRINGⁱ

10090.ENSMUSP00000030453

PTM databases

iPTMnetⁱ	Q9CZD0
PhosphoSitePlusⁱ	Q9CZD0

Proteomic databases

PaxDbⁱ	Q9CZD0
PRIDEⁱ	Q9CZD0

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	67096
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENSMUST00000030453; ENSMUSP00000030453; ENSMUSG00000028690
GeneIDⁱ	67096
KEGGⁱ	mmu:67096
UCSCⁱ	uc008uhe.1 mouse

Organism-specific databases

CTDⁱ	25974
MGIⁱ	MGI:1914346 Mmachc

Phylogenomic databases

eggNOGⁱ	ENOG410IKBC Eukaryota ENOG410XTCP LUCA
GeneTreeⁱ	ENSGT00390000003464
HOGENOMⁱ	HOG000231413
HOVERGENⁱ	HBG080267
InParanoidⁱ	Q9CZD0
KOⁱ	K14618
OMAⁱ	MEPQVAE
OrthoDBⁱ	EOG091G0GLW
PhylomeDBⁱ	Q9CZD0
TreeFamⁱ	TF332476

Enzyme and pathway databases

UniPathwayⁱ	UPA00148
Reactomeⁱ	R-MMU-196741 Cobalamin (Cbl, vitamin B12) transport and metabolism

Miscellaneous databases

ChiTaRSⁱ	Mmachc mouse
Protein Ontology More...PROⁱ	PR:Q9CZD0
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSMUSG00000028690 Expressed in 266 organ(s), highest expression level in gastrocnemius
Genevisibleⁱ	Q9CZD0 MM

Family and domain databases

CDDⁱ	cd12959 MMACHC-like, 1 hit
InterProⁱ	View protein in InterPro IPR032037 MMACHC
PANTHERⁱ	PTHR31457 PTHR31457, 1 hit
Pfamⁱ	View protein in Pfam PF16690 MMACHC, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	MMAC_MOUSE
Accessionⁱ	Q9CZD0Primary (citable) accession number: Q9CZD0 Secondary accession number(s): Q9D8S7
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	January 10, 2006
	Last sequence update:	January 10, 2006
	Last modified:	September 12, 2018
	This is version 101 of the entry and version 2 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
PATHWAY comments
Index of metabolic and biosynthesis pathways

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - Q9CZD0 (MMAC_MOUSE)

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Methylmalonic aciduria and homocystinuria type C protein homolog

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: adenosylcobalamin biosynthesis

Sites

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Other locations

Mitochondrion

Other locations

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Phylogenomic databases

Family and domain databases

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

Experimental Info

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

Pathwayⁱ: adenosylcobalamin biosynthesis

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Sequence cautionⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ