Article
Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca(2+) stabilization.
The EMBO journal - 15 Nov 2001
Chen C D, Huff M E, Matteson J, Page L, Phillips R, Kelly J W, Balch W E
Abstract excerpt
Hereditary familial amyloidosis of Finnish type (FAF) leading to amyloid in the peripheral and central nervous systems stems from deposition of a 71 residue fragment generated from the D187N/Y variants of plasma gelsolin by two sequential endoproteolytic events. We identify the protease accomplishing the first cleavage as furin, a proprotein convertase. Endoproteolysis of plasma gelsolin occurs in the trans-Golgi...
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