Low-level mitochondrial heteroplasmy modulates DNA replication, glucose metabolism and lifespan in mice

Misa Hirose, Paul Schilf, Yask Gupta, Kim Zarse, Axel Künstner, Anke Fähnrich, Hauke Busch, Junping Yin, Marvin N. Wright, Andreas Ziegler, Marie Vallier, Meriem Belheouane, John F Baines, Diethard Tautz, Kornelia Johann, Rebecca Oelkrug, Jens Mittag, Hendrik Lehnert, Alaa Othman, Olaf Jöhren, Markus Schwaninger, Cornelia Prehn, Jerzy Adamski, Kensuke Shima, Jan Rupp, Robert Häsler, Georg Fuellen, Rüdiger Köhling, Michael Ristow & Saleh M. Ibrahim; Scientific Reports volume 8, Article number: 5872 (2018) doi:10.1038/s41598-018-24290-6

One example of such disorders is Friedreich Ataxia (FA), which is resulted by the impaired expression of the nuclear genome encoded frataxin protein that affect OXPHOS function by mediating mitochondrial iron-sulphur-cluster biosynthesis. FA patients develop diabetes and exhibited decreased lifespan, and experimental evidence using different models showed that a frataxin knock-out cause diabetes in mice, and knocking down of the frataxin gene resulted in shorter lifespan in worms.

 Low-level mitochondrial heteroplasmy modulates DNA replication, glucose metabolism and lifespan in mice