Names & Taxonomy
- Uniprot ID:
- Q8IVH4
- Entry Name:
- MMAA_HUMAN
- Status:
- reviewed
- Protein Names:
- Methylmalonic aciduria type A protein, mitochondrial (EC 3.6.-.-)
- Gene Names:
- MMAA
- Gene Names Primary:
- MMAA
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 418
- Sequence:
- MPMLLPHPHQHFLKGLLRAPFRCYHFIFHSSTHLGSGIPCAQPFNSLGLHCTKWMLLSDGLKRKLCVQTTLKDHTEGLSDKEQRFVDKLYTGLIQGQRACLAEAITLVESTHSRKKELAQVLLQKVLLYHREQEQSNKGKPLAFRVGLSGPPGAGKSTFIEYFGKMLTERGHKLSVLAVDPSSCTSGGSLLGDKTRMTELSRDMNAYIRPSPTRGTLGGVTRTTNEAILLCEGAGYDIILIETVGVGQSEFAVADMVDMFVLLLPPAGGDELQGIKRGIIEMADLVAVTKSDGDLIVPARRIQAEYVSALKLLRKRSQVWKPKVIRISARSGEGISEMWDKMKDFQDLMLASGELTAKRRKQQKVWMWNLIQESVLEHFRTHPTVREQIPLLEQKVLIGALSPGLAADFLLKAFKSRD
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- Probable GTPase. May function as chaperone. May be involved in the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis.
- Pathway:
- Cofactor biosynthesis; adenosylcobalamin biosynthesis.
- Cross Reference Drug Bank:
- DB00115 DB00200
- Gene Ontology Go:
- mitochondrial matrix
GTP binding
GTPase activity
cellular lipid metabolic process
cobalamin biosynthetic process
cobalamin metabolic process
fatty acid beta-oxidation
short-chain fatty acid catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- cellular lipid metabolic process
cobalamin biosynthetic process
cobalamin metabolic process
fatty acid beta-oxidation
short-chain fatty acid catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- GTPase activity
GTP binding - Gene Ontology Cellular Component:
- mitochondrial matrix
- Keywords:
- 3D-structure
Chaperone
Complete proteome
Disease mutation
GTP-binding
Hydrolase
Mitochondrion
Nucleotide-binding
Polymorphism
Reference proteome
Transit peptide