{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T10:05:38Z","timestamp":1789985138583,"version":"4.0.1"},"reference-count":44,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2006,12,6]],"date-time":"2006-12-06T00:00:00Z","timestamp":1165363200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The FEBS Journal"],"published-print":{"date-parts":[[2007,1]]},"abstract":"<jats:p>Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease that usually manifests itself within the fifth decade. The most prominent symptoms are progressive ptosis, dysphagia, and proximal limb muscle weakness. The disorder is caused by trinucleotide (GCG) expansions in the N\u2010terminal part of the poly(A)\u2010binding protein 1 (PABPN1) that result in the extension of a 10\u2010alanine segment by up to seven more alanines. In patients, biopsy material displays intranuclear inclusions consisting primarily of PABPN1. Poly\u2003<jats:sc>l<\/jats:sc>\u2010alanine\u2010dependent fibril formation was studied using the recombinant N\u2010terminal domain of PABPN1. In the case of the protein fragment with the expanded poly\u2003<jats:sc>l<\/jats:sc>\u2010alanine sequence [N\u2010(+7)Ala], fibril formation could be induced by low amounts of fragmented fibrils serving as seeds. Besides homologous seeds, seeds derived from fibrils of the wild\u2010type fragment (N\u2010WT) also accelerated fibril formation of N\u2010(+7)Ala in a concentration\u2010dependent manner. Seed\u2010induced fibrillation of N\u2010WT was considerably slower than that of N\u2010(+7)Ala. Using atomic force microscopy, differences in fibril morphologies between N\u2010WT and N\u2010(+7)Ala were detected. Furthermore, fibrils of N\u2010WT showed a lower resistance against solubilization with the chaotropic agent guanidinium thiocyanate than those from N\u2010(+7)Ala. Our data clearly reveal biophysical differences between fibrils of the two variants that are likely caused by divergent fibril structures.<\/jats:p>","DOI":"10.1111\/j.1742-4658.2006.05595.x","type":"journal-article","created":{"date-parts":[[2006,12,6]],"date-time":"2006-12-06T17:09:36Z","timestamp":1165424976000},"page":"346-355","source":"Crossref","is-referenced-by-count":15,"title":["Effect of oculopharyngeal muscular dystrophy\u2010associated extension of seven alanines on the fibrillation properties of the N\u2010terminal domain of 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