Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD

Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD

Summary: Amyotrophic lateral sclerosis (ALS) manifests pathological changes in motor neurons and various other cell types. Compared to motor neurons, the contribution of the other cell types to the ALS phenotypes is understudied. G4C2 repeat expansion in C9ORF72 is the most common genetic cause of A...

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Bibliographic Details
Main Authors: Shuangxi Li, Zhihao Wu, Yu Li, Ishaq Tantray, Diego De Stefani, Andrea Mattarei, Gopinath Krishnan, Fen-Biao Gao, Hannes Vogel, Bingwei Lu
Format: Article
Language:English
Published: Elsevier 2020-08-01
Series:Cell Reports
Subjects:
C9-ALS/FTD
DPR
muscle
Opa1
MICOS
Mic27/Apool
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124720309748

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http://www.sciencedirect.com/science/article/pii/S2211124720309748

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