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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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| Main Authors: | , , , , , , , , , , , , , , , , , , , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
National Academy of Sciences
2014
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| Assuntos: | |
| 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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