Q8C7H1 · MMAA_MOUSE
- ProteinMethylmalonic aciduria type A homolog, mitochondrial
- GeneMmaa
- StatusUniProtKB reviewed (Swiss-Prot)
- Organism
- Amino acids415 (go to sequence)
- Protein existenceEvidence at protein level
- Annotation score3/5
Function
function
GTPase, binds and hydrolyzes GTP (By similarity).
Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis (By similarity).
Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT) (By similarity).
Plays a dual role as both a protectase and a reactivase for MMUT (By similarity).
Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl) (By similarity).
Additionally acts a reactivase by promoting the replacement of OH2Cbl by the active cofactor AdoCbl, restoring the activity of MMUT in the presence and hydrolysis of GTP (By similarity).
Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis (By similarity).
Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT) (By similarity).
Plays a dual role as both a protectase and a reactivase for MMUT (By similarity).
Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl) (By similarity).
Additionally acts a reactivase by promoting the replacement of OH2Cbl by the active cofactor AdoCbl, restoring the activity of MMUT in the presence and hydrolysis of GTP (By similarity).
Catalytic activity
- GTP + H2O = GDP + H+ + phosphate
Activity regulation
GTPase activity is stimulated by MMUT.
Features
Showing features for binding site.
GO annotations
Aspect | Term | |
---|---|---|
Cellular Component | cytoplasm | |
Cellular Component | cytosol | |
Cellular Component | mitochondrion | |
Molecular Function | ATP hydrolysis activity | |
Molecular Function | GTP binding | |
Molecular Function | GTPase activity | |
Molecular Function | identical protein binding | |
Molecular Function | protein homodimerization activity | |
Biological Process | cobalamin metabolic process |
Keywords
- Molecular function
- Ligand
Enzyme and pathway databases
Names & Taxonomy
Protein names
- Recommended nameMethylmalonic aciduria type A homolog, mitochondrial
- EC number
Gene names
Organism names
- Organism
- Strain
- Taxonomic lineageEukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus
Accessions
- Primary accessionQ8C7H1
- Secondary accessions
Proteomes
Organism-specific databases
Subcellular Location
PTM/Processing
Features
Showing features for transit peptide, chain.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Transit peptide | 1-62 | Mitochondrion | ||||
Sequence: MTISTLLLSPNRRLLTCLSRVPSPWLLHSSHPAPGPPGALPNCFGHHCTKRVLLSDGFRRTL | ||||||
Chain | PRO_0000002286 | 63-415 | Methylmalonic aciduria type A homolog, mitochondrial | |||
Sequence: CVQATLKDHTEGLSDKEQRFVDRLYTGLVKGQRACLAEAITLVESTHTRKRELAQVLLQRVLALQREQELRNQGKPLTFRVGLSGPPGAGKSTFIECFGKMLTEQGHRLSVLAVDPSSCTSGGSLLGDKTRMIELSRDMNAYIRPSPTSGTLGGVTRTTNEAIVLCEGGGYDIILIETVGVGQSEFAVADMVDMFVLLLPPAGGDELQGIKRGIIEMADLVVITKSDGDLIVPARRIQAEYVSALKLLRRRSEVWRPKVIRISARSGEGITEMWDTMREFQHQMLASGELAAKRQTQHKVWMWNLIQENVLEHFKTHPSIREQIPLMERKVLSGALSPGRAADLLLKAFKSRH |
Proteomic databases
PTM databases
Expression
Gene expression databases
Structure
Sequence
- Sequence statusComplete
- Sequence processingThe displayed sequence is further processed into a mature form.
- Length415
- Mass (Da)45,932
- Last updated2003-03-01 v1
- ChecksumA46967424CC4CC06
Computationally mapped potential isoform sequences
There is 1 potential isoform mapped to this entry
Entry | Entry name | Gene name | Length | ||
---|---|---|---|---|---|
A0A1B0GSL6 | A0A1B0GSL6_MOUSE | Mmaa | 155 |
Features
Showing features for sequence conflict.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Sequence conflict | 31 | in Ref. 2; AAH21954 | ||||
Sequence: H → P |
Keywords
- Technical term
Sequence databases
Nucleotide Sequence | Protein Sequence | Molecule Type | Status | |
---|---|---|---|---|
AK050255 EMBL· GenBank· DDBJ | BAC34149.1 EMBL· GenBank· DDBJ | mRNA | ||
BC021954 EMBL· GenBank· DDBJ | AAH21954.1 EMBL· GenBank· DDBJ | mRNA | ||
BC027398 EMBL· GenBank· DDBJ | AAH27398.1 EMBL· GenBank· DDBJ | mRNA |