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

作者：Bannwarth Sylvie; Figueroa Alexia; Fragaki Konstantina; Destroismaisons Laurie; Lacas Gervais Sandra; Lespinasse Francoise; Vandenbos Fanny; Pradelli Ludivine A; Ricci Jean Ehrland; Roetig Agnes; Michiels Jean Francois; Vande Velde Christine; Paquis Flucklinger Veronique^*

来源：Mitochondrion, 2012, 12(6): 654-665.

DOI：10.1016/j.mito.2012.07.111

• mtDNA instability
• DNA polymerase gamma
• Twinkle helicase
• Mitochondrial diseases

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.

• 出版日期2012-11