Article
The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease.
Brain : a journal of neurology - 1 Mar 2014
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
Abstract excerpt
The ganglioside-induced differentiation-associated protein 1 (GDAP1) is a mitochondrial fission factor and mutations in GDAP1 cause Charcot-Marie-Tooth disease. We found that Gdap1 knockout mice (Gdap1(-/-)), mimicking genetic alterations of patients suffering from severe forms of Charcot-Marie-Tooth disease, develop an age-related, hypomyelinating peripheral neuropathy. Ablation of Gdap1 expression in Schwann...
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