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Article

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

Proceedings of the National Academy of Sciences of the United States of America - 7 Jul 2009

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

Abstract excerpt

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 aberrant endoproteolytic cascade and the aging-associated extracellular amyloid deposition of FAF. Amyloidogenesis is...

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Identifiers

PMID: 19549824
DOI: 10.1073/pnas.0811753106
PMCID: PMC2708763

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Source receipt: pubmed, retrieved 7 Sept 2026.

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