Proteolytic cleavage of Ser52Pro variant transthyretin triggers its amyloid fibrillogenesis

The Ser52Pro variant of transthyretin (TTR) produces aggressive, highly penetrant, autosomal-dominant systemic amyloidosis in persons heterozygous for the causative mutation. Together with a minor quantity of full-length wild-type and variant TTR, the main component of the ex vivo fibrils was the re...

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Detalhes bibliográficos
Main Authors: Mangione, P. Patrizia, Porcari, Riccardo, Gillmore, Julian D., Pucci, Piero, Monti, Maria, Porcari, Mattia, Giorgetti, Sofia, Marchese, Loredana, Raimondi, Sara, Serpell, Louise C., Chen, Wenjie, Relini, Annalisa, Marcoux, Julien, Clatworthy, Innes R., Taylor, Graham W., Tennent, Glenys A., Robinson, Carol V., Hawkins, Philip N., Stoppini, Monica, Wood, Stephen P., Pepys, Mark B., Bellotti, Vittorio
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2014
Assuntos:
Biological Sciences
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3910611/
https://ncbi.nlm.nih.gov/pubmed/24474780
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1317488111
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