Skeletal-Muscle Metabolic Reprogramming in ALS-SOD1G93A Mice Predates Disease Onset and Is A Promising Therapeutic Target

Summary: Patients with ALS show, in addition to the loss of motor neurons in the spinal cord, brainstem, and cerebral cortex, an abnormal depletion of energy stores alongside hypermetabolism. In this study, we show that bioenergetic defects and muscle remodeling occur in skeletal muscle of the SOD1G...

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Main Authors: Silvia Scaricamazza, Illari Salvatori, Giacomo Giacovazzo, Jean Philippe Loeffler, Frederique Renè, Marco Rosina, Cyril Quessada, Daisy Proietti, Constantin Heil, Simona Rossi, Stefania Battistini, Fabio Giannini, Nila Volpi, Frederik J. Steyn, Shyuan T. Ngo, Elisabetta Ferraro, Luca Madaro, Roberto Coccurello, Cristiana Valle, Alberto Ferri
Format: Article
Language:English
Published: Elsevier 2020-05-01
Series:iScience
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2589004220302728