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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Detalles Bibliográficos
Autores principales: da Costa, Gonçalo, Gomes, Ricardo A., Guerreiro, Ana, Mateus, Élia, Monteiro, Estela, Barroso, Eduardo, Coelho, Ana V., Freire, Ana Ponces, Cordeiro, Carlos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3203866/
https://www.ncbi.nlm.nih.gov/pubmed/22053176
http://dx.doi.org/10.1371/journal.pone.0024850