Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in a Drosophila Model of Friedreich’s Ataxia

Edenharter O, Schneuwly S and Navarro JA (2018); Front. Mol. Neurosci. 11:38. doi: 10.3389/fnmol.2018.00038

We have demonstrated that Marf downregulation rescues the locomotion and the accumulation of lipid droplets triggered by targeted frataxin depletion in glia along with nervous system degeneration. The analysis of the mechanism underlying such recovery has allowed us to underscore that alterations in the ER biology are pivotal in the neurodegeneration in FRDA. Our results suggest that Marf and ER stress represent a hub in the neurodegenerative process of FRDA and uncover both as important elements substantially involved the FRDA pathology.

 Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in a Drosophila Model of Friedreich’s Ataxia