Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats

Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats

Abstract Mutations in ubiquilin2 (UBQLN2) have been linked to abnormal protein aggregation in amyotrophic lateral sclerosis (ALS). The mechanisms underlying UBQLN2-related neurodegenerative diseases remain unclear. Using a tetracycline-regulated gene expression system, the ALS-linked UBQLN2P497H mut...

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Bibliographic Details
Main Authors: Tianhong Chen, Bo Huang, Xinglong Shi, Limo Gao, Cao Huang
Format: Article
Language:English
Published: BMC 2018-11-01
Series:Acta Neuropathologica Communications
Subjects:
Amyotrophic lateral sclerosis
ALS
UBQLN2
P62
Motor neuron degeneration
Autophagy
Online Access:http://link.springer.com/article/10.1186/s40478-018-0627-9

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