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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Egile Nagusiak:	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
Formatua:	Artigo
Hizkuntza:	Inglês
Argitaratua:	Elsevier 2020-09-01
Saila:	Neurobiology of Disease
Gaiak:	Proteostasis Ubiquilin Ubiquitin proteasome system Amyotrophic lateral sclerosis Frontotemporal dementia Protein aggregation
Sarrera elektronikoa:	http://www.sciencedirect.com/science/article/pii/S0969996120302916
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