| Function | Isoform 1: Mitochondrial helicase involved in mtDNA replication and repair. Might have a role in mtDNA repair. 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. Preferentially unwinds DNA substrates with pre-existing 5'-and 3'- single-stranded tails but is also active on a 5'- flap substrate. Can dissociate the invading strand of immobile or mobile D-loop DNA structures irrespective of the single strand polarity of the third strand. In addition to its DNA strand separation activity, also has DNA strand annealing, DNA strand-exchange and DNA branch migration activities. Isoform 2: Lack DNA unwinding and ATP hydrolysis activities. Does not bind single-stranded or double-stranded DNA. |
| Protein Name | Twinkle Mtdna HelicaseProgressive External Ophthalmoplegia 1 ProteinT7 Gp4-Like Protein With Intramitochondrial Nucleoid LocalizationT7-Like Mitochondrial Dna HelicaseTwinkle Protein - Mitochondrial |
| Database Links | Reactome: R-HSA-2151201Reactome: R-HSA-9837999 |
| Cellular Localisation | Mitochondrion MatrixMitochondrion NucleoidMitochondrion Inner MembranePeripheral Membrane ProteinColocalizes With Mtdna In Mitochondrial NucleoidsA Nucleoproteins Complex Consisting Of A Number Of Copies Of Proteins Associated With MtdnaProbably Involved In Mtdna Maintenance And ExpressionAssociates With Phospholipid Membranes Via Electrostatic BindingPreferentially Associates With Membranes Enriched With CardiolipinA Lipid Abundant In The Mitochondrial Inner MembraneAtpase And Helicase Activity Is Enhanced By Binding To Lipid Membranes |
| Alternative Protein Names | Twinkle Mtdna Helicase proteinProgressive External Ophthalmoplegia 1 Protein proteinT7 Gp4-Like Protein With Intramitochondrial Nucleoid Localization proteinT7-Like Mitochondrial Dna Helicase proteinTwinkle Protein - Mitochondrial proteinTWNK proteinC10orf2 proteinPEO1 protein |
Information sourced from Uniprot.org
