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Items: 1 to 20 of 38


The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesis.

Jemt E, Farge G, Bäckström S, Holmlund T, Gustafsson CM, Falkenberg M.

Nucleic Acids Res. 2011 Nov;39(21):9238-49. doi: 10.1093/nar/gkr653. Epub 2011 Aug 12.


Single-stranded DNA binding proteins unwind the newly synthesized double-stranded DNA of model miniforks.

Delagoutte E, Heneman-Masurel A, Baldacci G.

Biochemistry. 2011 Feb 15;50(6):932-44. doi: 10.1021/bi101583e. Epub 2011 Jan 20.


Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein.

Oliveira MT, Kaguni LS.

PLoS One. 2010 Oct 28;5(10):e15379. doi: 10.1371/journal.pone.0015379.


Disease variants of the human mitochondrial DNA helicase encoded by C10orf2 differentially alter protein stability, nucleotide hydrolysis, and helicase activity.

Longley MJ, Humble MM, Sharief FS, Copeland WC.

J Biol Chem. 2010 Sep 24;285(39):29690-702. doi: 10.1074/jbc.M110.151795. Epub 2010 Jul 20.


The human mitochondrial replication fork in health and disease.

Wanrooij S, Falkenberg M.

Biochim Biophys Acta. 2010 Aug;1797(8):1378-88. doi: 10.1016/j.bbabio.2010.04.015. Epub 2010 Apr 22. Review.


Dynamic effects of cofactors and DNA on the oligomeric state of human mitochondrial DNA helicase.

Ziebarth TD, Gonzalez-Soltero R, Makowska-Grzyska MM, Núñez-Ramírez R, Carazo JM, Kaguni LS.

J Biol Chem. 2010 May 7;285(19):14639-47. doi: 10.1074/jbc.M109.099663. Epub 2010 Mar 8.


Eukaryotic DNA replication control: lock and load, then fire.

Remus D, Diffley JF.

Curr Opin Cell Biol. 2009 Dec;21(6):771-7. doi: 10.1016/ Epub 2009 Sep 18. Review.


Clinical and molecular aspects of diseases of mitochondrial DNA instability.

Mao CC, Holt IJ.

Chang Gung Med J. 2009 Jul-Aug;32(4):354-69. Review.


Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks.

Pohjoismäki JL, Goffart S, Tyynismaa H, Willcox S, Ide T, Kang D, Suomalainen A, Karhunen PJ, Griffith JD, Holt IJ, Jacobs HT.

J Biol Chem. 2009 Aug 7;284(32):21446-57. doi: 10.1074/jbc.M109.016600. Epub 2009 Jun 12.


Dual DNA unwinding activities of the Rothmund-Thomson syndrome protein, RECQ4.

Xu X, Liu Y.

EMBO J. 2009 Mar 4;28(5):568-77. doi: 10.1038/emboj.2009.13. Epub 2009 Jan 29.


Structure-function defects of the twinkle amino-terminal region in progressive external ophthalmoplegia.

Holmlund T, Farge G, Pande V, Korhonen J, Nilsson L, Falkenberg M.

Biochim Biophys Acta. 2009 Feb;1792(2):132-9. doi: 10.1016/j.bbadis.2008.11.009. Epub 2008 Nov 24.


Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA replication stalling.

Goffart S, Cooper HM, Tyynismaa H, Wanrooij S, Suomalainen A, Spelbrink JN.

Hum Mol Genet. 2009 Jan 15;18(2):328-40. doi: 10.1093/hmg/ddn359. Epub 2008 Oct 29.


Physiological and biochemical defects in carboxyl-terminal mutants of mitochondrial DNA helicase.

Matsushima Y, Farr CL, Fan L, Kaguni LS.

J Biol Chem. 2008 Aug 29;283(35):23964-71. doi: 10.1074/jbc.M803674200. Epub 2008 Jun 30.


Branch migration enzyme as a Brownian ratchet.

Rasnik I, Jeong YJ, McKinney SA, Rajagopal V, Patel SS, Ha T.

EMBO J. 2008 Jun 18;27(12):1727-35. doi: 10.1038/emboj.2008.106. Epub 2008 May 29.


Structure-function defects of the TWINKLE linker region in progressive external ophthalmoplegia.

Korhonen JA, Pande V, Holmlund T, Farge G, Pham XH, Nilsson L, Falkenberg M.

J Mol Biol. 2008 Mar 28;377(3):691-705. doi: 10.1016/j.jmb.2008.01.035. Epub 2008 Jan 26.


The N-terminal domain of TWINKLE contributes to single-stranded DNA binding and DNA helicase activities.

Farge G, Holmlund T, Khvorostova J, Rofougaran R, Hofer A, Falkenberg M.

Nucleic Acids Res. 2008 Feb;36(2):393-403. Epub 2007 Nov 26.


Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes.

Wanrooij S, Goffart S, Pohjoismäki JL, Yasukawa T, Spelbrink JN.

Nucleic Acids Res. 2007;35(10):3238-51. Epub 2007 Apr 22.


Modular architecture of the hexameric human mitochondrial DNA helicase.

Ziebarth TD, Farr CL, Kaguni LS.

J Mol Biol. 2007 Apr 13;367(5):1382-91. Epub 2007 Feb 7.


The accessory subunit B of DNA polymerase gamma is required for mitochondrial replisome function.

Farge G, Pham XH, Holmlund T, Khorostov I, Falkenberg M.

Nucleic Acids Res. 2007;35(3):902-11. Epub 2007 Jan 23.


Oligomeric states of bacteriophage T7 gene 4 primase/helicase.

Crampton DJ, Ohi M, Qimron U, Walz T, Richardson CC.

J Mol Biol. 2006 Jul 14;360(3):667-77. Epub 2006 May 30.

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