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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Detalhes bibliográficos
Publicado no:Neurobiol Dis
Main Authors: 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.
Formato: Artigo
Idioma:Inglês
Publicado em: 2020
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7504910/
https://ncbi.nlm.nih.gov/pubmed/32653673
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2020.105016
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