ataxin 8,ATXN8,C10orf2,IOSCA,MTDPS7,PEO,PEO1,PEOA3,PRLTS5,progressive external ophthalmoplegia 1 protein,SANDO,SCA8,T7 gp4-like protein with intramitochondrial nucleoid localization,T7 helicase-related protein with intramitochondrial nucleoid localization,T7-like mitochondrial DNA helicase,Twinkle mtDNA helicase,twinkle protein, mitochondrial,TWINL.
Involved in mitochondrial DNA (mtDNA) metabolism. Could function as an adenine nucleotide-dependent DNA helicase. Function inferred to be critical for lifetime maintenance of mtDNA integrity. In vitro, forms in combination with POLG, a processive replication machinery, which can use double-stranded DNA (dsDNA) as template to synthesize single-stranded DNA (ssDNA) molecules. May be a key regulator of mtDNA copy number in mammals.