Secretion of amyloidogenic gelsolin progressively compromises protein homeostasis leading to the intracellular aggregation of proteins

Familial amyloidosis of Finnish type (FAF) is a systemic amyloid disease associated with the deposition of proteolytic fragments of mutant (D187N/Y) plasma gelsolin. We report a mouse model of FAF featuring a muscle-specific promoter to drive D187N gelsolin synthesis. This model recapitulates the ab...

Descrizione completa

Salvato in:
Dettagli Bibliografici
Autori principali: Page, Lesley J., Suk, Ji Young, Bazhenova, Lyudmila, Fleming, Sheila M., Wood, Malcolm, Jiang, Yun, Guo, Ling T., Mizisin, Andrew P., Kisilevsky, Robert, Shelton, G. Diane, Balch, William E., Kelly, Jeffery W.
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2009
Soggetti:
Biological Sciences
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2708763/
https://ncbi.nlm.nih.gov/pubmed/19549824
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0811753106
Tags: Aggiungi Tag
Nessun Tag, puoi essere il primo ad aggiungerne! !

Documenti analoghi