Modeling UBQLN2-mediated neurodegenerative disease in mice: Shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity

Modeling UBQLN2-mediated neurodegenerative disease in mice: Shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity

The ubiquitin-binding proteasomal shuttle protein UBQLN2 is implicated in common neurodegenerative disorders due to its accumulation in disease-specific aggregates and, when mutated, directly causes familial frontotemporal dementia/amyotrophic lateral sclerosis (FTD/ALS). Like other proteins linked...

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Bibliographic Details
Main Authors: Lisa M. Sharkey, Stephanie S. Sandoval-Pistorius, Shannon J. Moore, Julia E. Gerson, Robert Komlo, Svetlana Fischer, Keyshla Y. Negron-Rios, Emily V. Crowley, Francisco Padron, Ronak Patel, Geoffrey G. Murphy, Henry L. Paulson
Format: Article
Language:English
Published: Elsevier 2020-09-01
Series:Neurobiology of Disease
Subjects:
Proteostasis
Ubiquilin
Ubiquitin proteasome system
Amyotrophic lateral sclerosis
Frontotemporal dementia
Protein aggregation
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996120302916

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http://www.sciencedirect.com/science/article/pii/S0969996120302916

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