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.

doi:10.1016/j.nbd.2020.105016

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Article

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.

Neurobiology of disease - 1 Sept 2020

Sharkey Lisa M, Sandoval-Pistorius Stephanie S, Moore Shannon J, Gerson Julia E, Komlo Robert, Fischer Svetlana, Negron-Rios Keyshla Y, Crowley Emily V, Padron Francisco, Patel Ronak, Murphy Geoffrey G, Paulson Henry L

Abstract excerpt

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 to FTD/ALS, UBQLN2 undergoes phase separation to form condensates. The relationship of UBQLN2 phase separation and...

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PMID: 32653673
DOI: 10.1016/j.nbd.2020.105016
PMCID: PMC7504910

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