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Items: 32

1.

ATAD3 controls mitochondrial cristae structure, influencing mtDNA replication and cholesterol levels in muscle.

Peralta S, Goffart S, Williams SL, Diaz F, Garcia S, Nissanka N, Area-Gomez E, Pohjoismäki J, Moraes CT.

J Cell Sci. 2018 Jun 13. pii: jcs.217075. doi: 10.1242/jcs.217075. [Epub ahead of print]

PMID:
29898916
2.

Preclinical Efficacy and Safety Evaluation of Hematopoietic Stem Cell Gene Therapy in a Mouse Model of MNGIE.

Yadak R, Cabrera-Pérez R, Torres-Torronteras J, Bugiani M, Haeck JC, Huston MW, Bogaerts E, Goffart S, Jacobs EH, Stok M, Leonardelli L, Biasco L, Verdijk RM, Bernsen MR, Ruijter G, Martí R, Wagemaker G, van Til NP, de Coo IFM.

Mol Ther Methods Clin Dev. 2018 Jan 8;8:152-165. doi: 10.1016/j.omtm.2018.01.001. eCollection 2018 Mar 16.

3.

Tissue specific differences in mitochondrial DNA maintenance and expression.

Herbers E, Kekäläinen NJ, Hangas A, Pohjoismäki JL, Goffart S.

Mitochondrion. 2018 Jan 12. pii: S1567-7249(17)30342-2. doi: 10.1016/j.mito.2018.01.004. [Epub ahead of print]

PMID:
29339192
4.

PrimPol is required for replication reinitiation after mtDNA damage.

Torregrosa-Muñumer R, Forslund JME, Goffart S, Pfeiffer A, Stojkovič G, Carvalho G, Al-Furoukh N, Blanco L, Wanrooij S, Pohjoismäki JLO.

Proc Natl Acad Sci U S A. 2017 Oct 24;114(43):11398-11403. doi: 10.1073/pnas.1705367114. Epub 2017 Oct 9.

5.

The role of mitochondria in cardiac development and protection.

Pohjoismäki JL, Goffart S.

Free Radic Biol Med. 2017 May;106:345-354. doi: 10.1016/j.freeradbiomed.2017.02.032. Epub 2017 Feb 17. Review.

PMID:
28216385
6.

Chemoproteomic, biochemical and pharmacological approaches in the discovery of inhibitors targeting human α/β-hydrolase domain containing 11 (ABHD11).

Navia-Paldanius D, Patel JZ, López Navarro M, Jakupović H, Goffart S, Pasonen-Seppänen S, Nevalainen TJ, Jääskeläinen T, Laitinen T, Laitinen JT, Savinainen JR.

Eur J Pharm Sci. 2016 Oct 10;93:253-63. doi: 10.1016/j.ejps.2016.08.031. Epub 2016 Aug 17.

PMID:
27544863
7.

Low doses of ultraviolet radiation and oxidative damage induce dramatic accumulation of mitochondrial DNA replication intermediates, fork regression, and replication initiation shift.

Torregrosa-Muñumer R, Goffart S, Haikonen JA, Pohjoismäki JL.

Mol Biol Cell. 2015 Nov 15;26(23):4197-208. doi: 10.1091/mbc.E15-06-0390. Epub 2015 Sep 23.

8.

Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species.

Pohjoismäki JL, Williams SL, Boettger T, Goffart S, Kim J, Suomalainen A, Moraes CT, Braun T.

Proc Natl Acad Sci U S A. 2013 Nov 26;110(48):19408-13. doi: 10.1073/pnas.1303046110. Epub 2013 Nov 11.

9.

Binding to G-quadruplex RNA activates the mitochondrial GTPase NOA1.

Al-Furoukh N, Goffart S, Szibor M, Wanrooij S, Braun T.

Biochim Biophys Acta. 2013 Dec;1833(12):2933-2942. doi: 10.1016/j.bbamcr.2013.07.022. Epub 2013 Aug 8.

10.

Oxidative stress during mitochondrial biogenesis compromises mtDNA integrity in growing hearts and induces a global DNA repair response.

Pohjoismäki JL, Boettger T, Liu Z, Goffart S, Szibor M, Braun T.

Nucleic Acids Res. 2012 Aug;40(14):6595-607. doi: 10.1093/nar/gks301. Epub 2012 Apr 15.

11.

Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells.

Pohjoismäki JL, Goffart S, Spelbrink JN.

Mitochondrion. 2011 Jul;11(4):630-4. doi: 10.1016/j.mito.2011.04.002. Epub 2011 Apr 20.

PMID:
21540127
12.

Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA.

Pohjoismäki JL, Goffart S.

Bioessays. 2011 Apr;33(4):290-9. doi: 10.1002/bies.201000137. Epub 2011 Feb 2. Review.

PMID:
21290399
13.

Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia.

Atanassova N, Fusté JM, Wanrooij S, Macao B, Goffart S, Bäckström S, Farge G, Khvorostov I, Larsson NG, Spelbrink JN, Falkenberg M.

Hum Mol Genet. 2011 Mar 15;20(6):1212-23. doi: 10.1093/hmg/ddq565. Epub 2011 Jan 12.

PMID:
21228000
14.

Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number.

Pohjoismäki JL, Goffart S, Taylor RW, Turnbull DM, Suomalainen A, Jacobs HT, Karhunen PJ.

PLoS One. 2010 May 3;5(5):e10426. doi: 10.1371/journal.pone.0010426.

15.

Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid.

Pohjoismäki JL, Holmes JB, Wood SR, Yang MY, Yasukawa T, Reyes A, Bailey LJ, Cluett TJ, Goffart S, Willcox S, Rigby RE, Jackson AP, Spelbrink JN, Griffith JD, Crouch RJ, Jacobs HT, Holt IJ.

J Mol Biol. 2010 Apr 16;397(5):1144-55. doi: 10.1016/j.jmb.2010.02.029. Epub 2010 Feb 23.

16.

Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA.

Dalla Rosa I, Goffart S, Wurm M, Wiek C, Essmann F, Sobek S, Schroeder P, Zhang H, Krutmann J, Hanenberg H, Schulze-Osthoff K, Mielke C, Pommier Y, Boege F, Christensen MO.

Nucleic Acids Res. 2009 Oct;37(19):6414-28. doi: 10.1093/nar/gkp708. Epub 2009 Aug 31.

17.

The accessory subunit of mitochondrial DNA polymerase gamma determines the DNA content of mitochondrial nucleoids in human cultured cells.

Di Re M, Sembongi H, He J, Reyes A, Yasukawa T, Martinsson P, Bailey LJ, Goffart S, Boyd-Kirkup JD, Wong TS, Fersht AR, Spelbrink JN, Holt IJ.

Nucleic Acids Res. 2009 Sep;37(17):5701-13. doi: 10.1093/nar/gkp614. Epub 2009 Jul 22.

18.

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.

19.

Inducible expression in human cells, purification, and in vitro assays for the mitochondrial DNA helicase Twinkle.

Goffart S, Spelbrink H.

Methods Mol Biol. 2009;554:103-19. doi: 10.1007/978-1-59745-521-3_7.

PMID:
19513670
20.

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.

21.

Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.

Hakonen AH, Goffart S, Marjavaara S, Paetau A, Cooper H, Mattila K, Lampinen M, Sajantila A, Lönnqvist T, Spelbrink JN, Suomalainen A.

Hum Mol Genet. 2008 Dec 1;17(23):3822-35. doi: 10.1093/hmg/ddn280. Epub 2008 Sep 5.

PMID:
18775955
22.

The mitochondria of cultured mammalian cells: II. Expression and visualization of exogenous proteins in fixed and live cells.

Goffart S, Martinsson P, Malka F, Rojo M, Spelbrink JN.

Methods Mol Biol. 2007;372:17-32. doi: 10.1007/978-1-59745-365-3_2.

PMID:
18314715
23.

The mitochondria of cultured mammalian cells: I. Analysis by immunofluorescence microscopy, histochemistry, subcellular fractionation, and cell fusion.

Malka F, Auré K, Goffart S, Spelbrink JN, Rojo M.

Methods Mol Biol. 2007;372:3-16. doi: 10.1007/978-1-59745-365-3_1.

PMID:
18314714
24.

CREB-1alpha is recruited to and mediates upregulation of the cytochrome c promoter during enhanced mitochondrial biogenesis accompanying skeletal muscle differentiation.

Franko A, Mayer S, Thiel G, Mercy L, Arnould T, Hornig-Do HT, Wiesner RJ, Goffart S.

Mol Cell Biol. 2008 Apr;28(7):2446-59. doi: 10.1128/MCB.00980-07. Epub 2008 Jan 28.

25.

Twinkle helicase (PEO1) gene mutation causes mitochondrial DNA depletion.

Sarzi E, Goffart S, Serre V, Chrétien D, Slama A, Munnich A, Spelbrink JN, Rötig A.

Ann Neurol. 2007 Dec;62(6):579-87.

PMID:
17722119
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.

27.

Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells.

Pohjoismäki JL, Wanrooij S, Hyvärinen AK, Goffart S, Holt IJ, Spelbrink JN, Jacobs HT.

Nucleic Acids Res. 2006;34(20):5815-28. Epub 2006 Oct 24.

28.

Alpha-actinin 4 and BAT1 interaction with the cytochrome c promoter upon skeletal muscle differentiation.

Goffart S, Franko A, Clemen CS, Wiesner RJ.

Curr Genet. 2006 Feb;49(2):125-35. Epub 2005 Dec 6.

PMID:
16331456
29.
30.

Regulation of mitochondrial proliferation in the heart: power-plant failure contributes to cardiac failure in hypertrophy.

Goffart S, von Kleist-Retzow JC, Wiesner RJ.

Cardiovasc Res. 2004 Nov 1;64(2):198-207. Review.

PMID:
15485678
31.

Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis.

Goffart S, Wiesner RJ.

Exp Physiol. 2003 Jan;88(1):33-40. Review.

PMID:
12525853
32.

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