The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease

Niemann, Axel ; Huber, Nina ; Wagner, Konstanze M. ; Somandin, Christian ; Horn, Michael ; Lebrun-Julien, Frédéric ; Angst, Brigitte ; Pereira, Jorge A. ; Halfter, Hartmut ; Welzl, Hans ; Feltri, M. Laura ; Wrabetz, Lawrence ; Young, Peter ; Wessig, Carsten ; Toyka, Klaus V. ; Suter, Ueli

In: Brain, 2014, vol. 137, no. 3, p. 668-682

Add to personal list
    Mutations in the mitochondrial fission factor GDAP1 are associated with severe peripheral neuropathies, but why the CNS remains unaffected is unclear. Using a Gdap1−/− mouse, Niemann et al. demonstrate that a CNS-expressed Gdap1 paralogue changes its subcellular localisation under oxidative stress conditions to also act as a mitochondrial fission factor