The human MSH5 (MutS Homolog 5) protein localizes to mitochondria and protects the mitochondrial genome from oxidative damage

被引：18

作者：

Bannwarth, Sylvie ^{[1
,2
]}

Figueroa, Alexia ^{[1
,2
]}

Fragaki, Konstantina ^{[1
,2
]}

Destroismaisons, Laurie ^{[3
,4
]}

Lacas-Gervais, Sandra ^{[5
]}

Lespinasse, Francoise ^{[1
]}

Vandenbos, Fanny ^{[6
]}

Pradelli, Ludivine A. ^{[7
]}

Ricci, Jean-Ehrland ^{[7
]}

Roetig, Agnes ^{[8
]}

Michiels, Jean-Francois ^{[6
]}

Vande Velde, Christine ^{[3
,4
]}

Paquis-Flucklinger, Veronique ^{[1
,2
]}

机构：

[1] Fac Med Nice, IRCAN, CNRS UMR 7284, UNS,Inserm U1081, F-06107 Nice 2, France

[2] CHU Nice, Hop Archet 2, Serv Genet Med, Nice, France

[3] Univ Montreal, CHUM, Ctr Rech, Ctr Excellence Neurom, Montreal, PQ H2L 4M1, Canada

[4] Univ Montreal, Dept Med, Montreal, PQ H2L 4M1, Canada

[5] Univ Nice Sophia Antipolis, Fac Sci, Ctr Commun Microscopie Elect Appl, Nice, France

[6] CHU Nice, Hop Louis Pasteur, Serv Neuropathol, Nice, France

[7] Equipe 3 AVENIR, C3M, INSERM, U895, F-06204 Nice 3, France

[8] Univ Paris 05, Hop Necker Enfants Malad, INSERM, U781, F-75015 Paris, France

来源：

MITOCHONDRION | 2012年 / 12卷 / 06期

基金：

加拿大健康研究院;

关键词：

mtDNA instability; DNA polymerase gamma; Twinkle helicase; Mitochondrial diseases; MISMATCH-REPAIR SYSTEM; SACCHAROMYCES-CEREVISIAE; MAMMALIAN MITOCHONDRIA; DNA-DAMAGE; HUMAN-CELLS; GENE; MTDNA; DELETIONS; MEIOSIS; HMSH5;

D O I：

10.1016/j.mito.2012.07.111

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

MutS homologs play a central role in maintaining genetic stability. We show that MSH5 (MutS Homolog 5) is localized into the mitochondria of germ and somatic cells. This protein binds to mtDNA and interacts with the Twinkle helicase and the DNA polymerase gamma. hMSH5 stimulates mtDNA repair in response to DNA damage induced by oxidative stress. Furthermore, we observed a subsarcolemmal accumulation of hMSH5 in COX negative muscle fibers of patients presenting a mitochondrial myopathy. We report a novel localization for hMSH5 suggesting that this protein may have functions other than those known in meiotic recombination. (C) 2012 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

引用

页码：654 / 665

页数：12

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