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Mutant UBQLN2(P497H) in motor neurons leads to ALS-like phenotypes and defective autophagy in rats

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 UBQLN2(P497H) mutant was...

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Detalles Bibliográficos
Autores principales: Chen, Tianhong, Huang, Bo, Shi, Xinglong, Gao, Limo, Huang, Cao
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6225656/
https://www.ncbi.nlm.nih.gov/pubmed/30409191
http://dx.doi.org/10.1186/s40478-018-0627-9