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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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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 |