| IED ID | IndEnz0002016617 |
| Enzyme Type ID | protease016617 |
| Protein Name |
AFG3-like protein 2 EC 3.4.24.- |
| Gene Name | Afg3l2 |
| Organism | Mus musculus (Mouse) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Glires (Rodents and rabbits) Rodentia Myomorpha (mice and others) Muroidea Muridae Murinae Mus Mus Mus musculus (Mouse) |
| Enzyme Sequence | MAHRCLLLWSRGGCRRGLPPLLVPRGCLGPDRRPCLRTLYQYATVQTASSRRSLLRDVIAAYQRFCSRPPKGFEKYFPNGKNGKKASEPKEAVGEKKEPQPSGPQPSGGAGGGGGKRRGKKEDSHWWSRFQKGDFPWDDKDFRMYFLWTALFWGGVMIYFVFKSSGREITWKDFVNNYLSKGVVDRLEVVNKRFVRVTFTPGKTPVDGQYVWFNIGSVDTFERNLETLQQELGIEGENRVPVVYIAESDGSFLLSMLPTVLIIAFLLYTIRRGPAGIGRTGRGMGGLFSVGETTAKVLKDEIDVKFKDVAGCEEAKLEIMEFVNFLKNPKQYQDLGAKIPKGAILTGPPGTGKTLLAKATAGEANVPFITVSGSEFLEMFVGVGPARVRDLFALARKNAPCILFIDEIDAVGRKRGRGNFGGQSEQENTLNQLLVEMDGFNTTTNVVILAGTNRPDILDPALLRPGRFDRQIFIGPPDIKGRASIFKVHLRPLKLDSALEKDKLARKLASLTPGFSGADVANVCNEAALIAARHLSDAINEKHFEQAIERVIGGLEKKTQVLQPEEKKTVAYHEAGHAVAGWYLEHADPLLKVSIIPRGKGLGYAQYLPKEQYLYTKEQLLDRMCMTLGGRVSEEIFFGRITTGAQDDLRKVTQSAYAQIVQFGMNEKVGQISFDLPRQGDMVLEKPYSEATARMIDDEVRILISDAYRRTVALLTEKKADVEKVALLLLEKEVLDKNDMVQLLGPRPFTEKSTYEEFVEGTGSLDEDTSLPEGLQDWNKEREKEEKKEKEKEEPLNEKVVS |
| Enzyme Length | 802 |
| Uniprot Accession Number | Q8JZQ2 |
| Absorption | |
| Active Site | ACT_SITE 574; /evidence=ECO:0000250|UniProtKB:Q9WZ49 |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | |
| EC Number | 3.4.24.- |
| Enzyme Function | FUNCTION: ATP-dependent protease which is essential for axonal and neuron development (PubMed:18337413, PubMed:27642048). In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (By similarity). Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form. Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (By similarity). Involved in the regulation of OMA1-dependent processing of OPA1 (By similarity). {ECO:0000250|UniProtKB:Q9Y4W6, ECO:0000269|PubMed:18337413, ECO:0000269|PubMed:27642048}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | NP_BIND 347..354; /note=ATP; /evidence=ECO:0000255 |
| Features | Active site (1); Chain (1); Compositional bias (1); Metal binding (3); Modified residue (1); Mutagenesis (1); Natural variant (1); Nucleotide binding (1); Propeptide (1); Region (2); Transit peptide (1); Transmembrane (2) |
| Keywords | ATP-binding;Direct protein sequencing;Disease variant;Hydrolase;Membrane;Metal-binding;Metalloprotease;Mitochondrion;Mitochondrion inner membrane;Nucleotide-binding;Protease;Reference proteome;Transit peptide;Transmembrane;Transmembrane helix;Zinc |
| Interact With | |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:19656850, ECO:0000269|PubMed:24856930}; Multi-pass membrane protein {ECO:0000255}. |
| Modified Residue | MOD_RES 116; /note=N6-succinyllysine; /evidence=ECO:0007744|PubMed:23806337 |
| Post Translational Modification | PTM: Upon import into the mitochondrion, the N-terminal transit peptide is cleaved to generate an intermediate form which undergoes autocatalytic proteolytic processing to generate the proteolytically active mature form. {ECO:0000269|PubMed:19656850}. |
| Signal Peptide | |
| Structure 3D | |
| Cross Reference PDB | - |
| Mapped Pubmed ID | 10498884; 11217851; 12466851; 12865426; 14610273; 16033648; 16141072; 16615898; 18614015; 19022416; 19289403; 19625515; 20038678; 21267068; 21677750; 22678058; 23041622; 24681487; 25485680; 26504172; 27689697; 27911893; 2917690; 30389403; 30910913; 30989755; 31781336; 8642395; 9455973; 9678332; |
| Motif | |
| Gene Encoded By | |
| Mass | 89,519 |
| Kinetics | |
| Metal Binding | METAL 573; /note=Zinc; catalytic; /evidence=ECO:0000250|UniProtKB:Q9WZ49; METAL 577; /note=Zinc; catalytic; /evidence=ECO:0000250|UniProtKB:Q9WZ49; METAL 648; /note=Zinc; catalytic; /evidence=ECO:0000250|UniProtKB:Q9WZ49 |
| Rhea ID | |
| Cross Reference Brenda | 3.4.24.B18; |