Article
ALS-causing mutations differentially affect PGC-1α expression and function in the brain vs. peripheral tissues.
Neurobiology of disease - 1 Jan 2017
Bayer Hanna, Lang Kerstin, Buck Eva, Higelin Julia, Barteczko Lara, Pasquarelli Noemi, Sprissler Jasmin, Lucas Tanja, Holzmann Karlheinz, Demestre Maria, Lindenberg Katrin S, Danzer Karin M, Boeckers Tobias, Ludolph Albert C, Dupuis Luc, Weydt Patrick, Witting Anke
Abstract excerpt
BACKGROUND: Monogenetic forms of amyotrophic lateral sclerosis (ALS) offer an opportunity for unraveling the molecular mechanisms underlying this devastating neurodegenerative disorder. In order to identify a link between ALS-related metabolic changes and neurodegeneration, we investigated whether ALS-causing mutations interfere with the peripheral and brain-specific expression and signaling of the metabolic...
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