Article
Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria.
Nature communications - 23 Mar 2018
Matic Stanka, Jiang Min, Nicholls Thomas J, Uhler Jay P, Dirksen-Schwanenland Caren, Polosa Paola Loguercio, Simard Marie-Lune, Li Xinping, Atanassov Ilian, Rackham Oliver, Filipovska Aleksandra, Stewart James B, Falkenberg Maria, Larsson Nils-Göran, Milenkovic Dusanka
Abstract excerpt
Replication of mammalian mitochondrial DNA (mtDNA) is an essential process that requires high fidelity and control at multiple levels to ensure proper mitochondrial function. Mutations in the mitochondrial genome maintenance exonuclease 1 (MGME1) gene were recently reported in mitochondrial disease patients. Here, to study disease pathophysiology, we generated Mgme1 knockout mice and report that homozygous...
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