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MIRAGE
Mitochondrial inner membrane m-AAA protease component paraplegin
Summary
- UniProt ID
- Q9UQ90
- Gene Symbol
-
- SPG7
- CAR
- CMAR
- PGN
- Organism
- Homo sapiens (human)
Annotation
- Keyword
-
- 3D-structure
- ATP-binding
- Alternative splicing
- Disease variant
- Hereditary spastic paraplegia
- Metal-binding
- Metalloprotease
- Mitochondrion inner membrane
- Nitration
- Osteogenesis imperfecta
- Reference proteome
- Transit peptide
- Transmembrane helix
- Zinc
- Gene Ontology (GO)
| GO Term |
|---|
| mitochondrion |
| mitochondrial inner membrane |
| axon cytoplasm |
| mitochondrial permeability transition pore complex |
| m-AAA complex |
| GO Term |
|---|
| metalloendopeptidase activity |
| ATP binding |
| peptidase activity |
| ATP hydrolysis activity |
| ATP-dependent peptidase activity |
| zinc ion binding |
| unfolded protein binding |
Sequence
MAVLLLLLRALRRGPGPGPRPLWGPGPAWSPGFPARPGRGRPYMASRPPGDLAEAGGRALQSLQLRLLTPTFEGINGLLLKQHLVQNPVRLWQLLGGTFYFNTSRLKQKNKEKDKSKGKAPEEDEEERRRRERDDQMYRERLRTLLVIAVVMSLLNALSTSGGSISWNDFVHEMLAKGEVQRVQVVPESDVVEVYLHPGAVVFGRPRLALMYRMQVANIDKFEEKLRAAEDELNIEAKDRIPVSYKRTGFFGNALYSVGMTAVGLAILWYVFRLAGMTGREGGFSAFNQLKMARFTIVDGKMGKGVSFKDVAGMHEAKLEVREFVDYLKSPERFLQLGAKVPKGALLLGPPGCGKTLLAKAVATEAQVPFLAMAGPEFVEVIGGLGAARVRSLFKEARARAPCIVYIDEIDAVGKKRSTTMSGFSNTEEEQTLNQLLVEMDGMGTTDHVIVLASTNRADILDGALMRPGRLDRHVFIDLPTLQERREIFEQHLKSLKLTQSSTFYSQRLAELTPGFSGADIANICNEAALHAAREGHTSVHTLNFEYAVERVLAGTAKKSKILSKEEQKVVAFHESGHALVGWMLEHTEAVMKVSITPRTNAALGFAQMLPRDQHLFTKEQLFERMCMALGGRASEALSFNEVTSGAQDDLRKVTRIAYSMVKQFGMAPGIGPISFPEAQEGLMGIGRRPFSQGLQQMMDHEARLLVAKAYRHTEKVLQDNLDKLQALANALLEKEVINYEDIEALIGPPPHGPKKMIAPQRWIDAQREKQDLGEEETEETQQPPLGGEEPTWPK
Glycosylation Sites
| Position | Description | PubMed ID | GlyTouCan ID | Source |
|---|---|---|---|---|
| 792 |
|
|
Feature
: Glycosylation Site from GlyGen
: Glycosylation Site from UniProt
Pathway
| Pathway Name | Organism |
|---|---|
| Mitochondrial protein degradation | Homo sapiens |
| Processing of SMDT1 | Homo sapiens |
