Twinkle mtDNA helicase

Definition:

[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.

References:

[1]. Jenny A Korhonen, et al. Reconstitution of a minimal mtDNA replisome in vitro. EMBO J. 2004 Jun 16;23(12):2423-9. [Content Brief]

[2]. Tawn D Ziebarth, et al. Modular architecture of the hexameric human mitochondrial DNA helicase. J Mol Biol. 2007 Apr 13;367(5):1382-91. [Content Brief]

[3]. Doyel Sen, et al. Human mitochondrial DNA helicase TWINKLE is both an unwinding and annealing helicase. J Biol Chem. 2012 Apr 27;287(18):14545-56. [Content Brief]

[4]. Doyel Sen, et al. Homologous DNA strand exchange activity of the human mitochondrial DNA helicase TWINKLE. Nucleic Acids Res. 2016 May 19;44(9):4200-10. [Content Brief]

[5]. Pablo Fernández-Millán, et al. The hexameric structure of the human mitochondrial replicative helicase Twinkle. Nucleic Acids Res. 2015 Apr 30;43(8):4284-95. [Content Brief]

[6]. Géraldine Farge, et al. The N-terminal domain of TWINKLE contributes to single-stranded DNA binding and DNA helicase activities. Nucleic Acids Res. 2008 Feb;36(2):393-403. [Content Brief]

[7]. Jenny A Korhonen, et al. TWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein. J Biol Chem. 2003 Dec 5;278(49):48627-32. [Content Brief]

[8]. Steffi Goffart, et al. Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA replication stalling. Hum Mol Genet. 2009 Jan 15;18(2):328-40. [Content Brief]

[9]. Irfan Khan, et al. Biochemical Characterization of the Human Mitochondrial Replicative Twinkle Helicase: SUBSTRATE SPECIFICITY, DNA BRANCH MIGRATION, AND ABILITY TO OVERCOME BLOCKADES TO DNA UNWINDING. J Biol Chem. 2016 Jul 1;291(27):14324-14339. [Content Brief]

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