Proteolytic cleavage of Ser52Pro variant transthyretin triggers its amyloid fibrillogenesis.

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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Bibliographic Details
Main Authors: Mangione, P, Porcari, R, Gillmore, J, Pucci, P, Monti, M, Porcari, M, Giorgetti, S, Marchese, L, Raimondi, S, Serpell, L, Chen, W, Relini, A, Marcoux, J, Clatworthy, I, Taylor, G, Tennent, G, Robinson, C, Hawkins, P, Stoppini, M, Wood, S, Pepys, M, Bellotti, V
Format: Journal article
Language:English
Published: 2014

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