Article
Two different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich's ataxia.
Disease models & mechanisms - 1 Jun 2016
Mollá Belén, Riveiro Fátima, Bolinches-Amorós Arantxa, Muñoz-Lasso Diana C, Palau Francesc, González-Cabo Pilar
Abstract excerpt
Frataxin (FXN) deficiency causes Friedreich's ataxia (FRDA), a multisystem disorder with neurological and non-neurological symptoms. FRDA pathophysiology combines developmental and degenerative processes of dorsal root ganglia (DRG), sensory nerves, dorsal columns and other central nervous structures. A dying-back mechanism has been proposed to explain the peripheral neuropathy and neuropathology. In addition,...
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