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...

Fuld beskrivelse

Na minha lista:
Bibliografiske detaljer
Main Authors: 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.
Format: Artigo
Sprog:Inglês
Udgivet: National Academy of Sciences 2009
Fag:
Biological Sciences
Online adgang: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: Tilføj Tag
Ingen Tags, Vær først til at tagge denne postø!

Lignende værker