Beyond genetic factors in familial amyloidotic polyneuropathy: protein glycation and the loss of fibrinogen's chaperone activity.

Familial amyloidotic polyneuropathy (FAP) is a systemic conformational disease characterized by extracellular amyloid fibril formation from plasma transthyretin (TTR). This is a crippling, fatal disease for which liver transplantation is the only effective therapy. More than 80 TTR point mutations a...

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Bibliographic Details
Main Authors: Gonçalo da Costa, Ricardo A Gomes, Ana Guerreiro, Élia Mateus, Estela Monteiro, Eduardo Barroso, Ana V Coelho, Ana Ponces Freire, Carlos Cordeiro
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3203866?pdf=render