| IED ID | IndEnz0002000422 |
| Enzyme Type ID | protease000422 |
| Protein Name |
Twinkle mtDNA helicase EC 3.6.4.12 Progressive external ophthalmoplegia 1 protein homolog T7 gp4-like protein with intramitochondrial nucleoid localization T7-like mitochondrial DNA helicase Twinkle protein, mitochondrial |
| Gene Name | Twnk Peo1 |
| 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 | MWLLLRRAYPLRILLPLRGEWVGRRGLPRSLAPGPPRRRYRKEALPALEMPVSPVTTTEIRQYLRAHGIPFQDGHSCLRAPSPFVVSSDIKNEKKDAPTSFCLFIDKTTGHFLCMTSLAEGSWEDLQASVEGRGDGAKEGVLLREGPEAEVREEVLRIWNRAIPLWELPDPEEAQLARVMFGLTKVTDDTLRRFSVRYLRSARSLVFPWFTPGSSGLRGLKLLGAEGQENGVQYVETTIPRPGVYHNLFGLPLISRRDTEVVVTSRELDSLALSQSTGLPTLSLPRGTVCLPPALLPYLEQFRRIVFWLGDDLRSWEAAKLFARKLNPKRCSLVRPGNQQPRPLEALNQGLSLPRILRTALPAWHKSIVSFRQLREEVLGELSNVEQAAGVRWSRFPDLNRLLKGHRKGELTVFTGPTGSGKTTFISEYALDLCTQGVNTLWGSFEISNVRLARVMLTQFAVTRLEEQLDKYEEWADRFEDLPLYFMTFHGQQSIRSVIDTMQHAVYVYDVCHVVIDNLQFMMGHEQLSSDRIAAQDYIVGAFRKFATDNSCHVTLVIHPRKEDDDKELQTASIFGSAKASQEADNVLILQDRKLVTGPGKRYLQVSKNRFDGDVGVFPLEFNKNSLTFSIPPKSKARLKKIKDDNGLVAKKSSSGKKGAAHQNPEICLGQDPSPAQPDTSKSSG |
| Enzyme Length | 685 |
| Uniprot Accession Number | Q8CIW5 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | CATALYTIC ACTIVITY: Reaction=ATP + H2O = ADP + H(+) + phosphate; Xref=Rhea:RHEA:13065, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; EC=3.6.4.12; Evidence={ECO:0000250|UniProtKB:Q96RR1}; |
| DNA Binding | |
| EC Number | 3.6.4.12 |
| Enzyme Function | FUNCTION: Mitochondrial helicase involved in mtDNA replication and repair (By similarity). Might have a role in mtDNA repair (By similarity). Has DNA strand separation activity needed to form a processive replication fork for leading strand synthesis which is catalyzed by the formation of a replisome complex with POLG and mtSDB (By similarity). Preferentially unwinds DNA substrates with pre-existing 5'-and 3'- single-stranded tails but is also active on a 5'- flap substrate (By similarity). Can dissociate the invading strand of immobile or mobile D-loop DNA structures irrespective of the single strand polarity of the third strand (By similarity). In addition to its DNA strand separation activity, also has DNA strand annealing, DNA strand-exchange and DNA branch migration activities (By similarity). {ECO:0000250|UniProtKB:Q96RR1}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | NP_BIND 416..423; /note=ATP; /evidence=ECO:0000255|PROSITE-ProRule:PRU00596 |
| Features | Alternative sequence (2); Chain (1); Coiled coil (1); Domain (1); Nucleotide binding (1); Region (5); Transit peptide (1) |
| Keywords | ATP-binding;Alternative splicing;Coiled coil;DNA replication;Helicase;Hydrolase;Mitochondrion;Mitochondrion nucleoid;Nucleotide-binding;Reference proteome;Transit peptide |
| Interact With | |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion matrix, mitochondrion nucleoid {ECO:0000250|UniProtKB:Q96RR1}. Note=Colocalizes with mtDNA in mitochondrial nucleoids, a nucleoproteins complex consisting of a number of copies of proteins associated with mtDNA, probably involved in mtDNA maintenance and expression. {ECO:0000250|UniProtKB:Q96RR1}. |
| Modified Residue | |
| Post Translational Modification | |
| Signal Peptide | |
| Structure 3D | |
| Cross Reference PDB | - |
| Mapped Pubmed ID | 10725249; 11431692; 14610273; 16301523; 18614015; 18799693; 18971204; 19620350; 22012983; 22949510; 23393161; 24076137; 25822152; 26253742; 26924217; 27342873; 29132502; 32737078; |
| Motif | |
| Gene Encoded By | |
| Mass | 76,993 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | RHEA:13065 |
| Cross Reference Brenda | 3.6.4.12; |