{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T04:52:37Z","timestamp":1790484757317,"version":"4.1.0"},"reference-count":42,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2009,1,1]],"date-time":"2009-01-01T00:00:00Z","timestamp":1230768000000},"content-version":"vor","delay-in-days":1858,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Protein Science"],"published-print":{"date-parts":[[2003,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The nuclear poly(A) binding protein (PABPN1) stimulates poly(A) polymerase and controls the lengths of poly(A) tails during pre\u2010mRNA processing. The wild\u2010type protein possesses 10 consecutive Ala residues immediately after the start methionine. Trinucleotide expansions in the coding sequence result in an extension of the Ala stretch to maximal 17 Ala residues in total. Individuals carrying the trinucleotide expansions suffer from oculopharyngeal muscular dystrophy (OPMD). Intranuclear inclusions consisting predominantly of PABPN1 have been recognized as a pathological hallmark of the genetic disorder. To elucidate the molecular events that lead to disease, recombinant PABPN1, and N\u2010terminal fragments of the protein with varying poly\u2010<jats:sc>l<\/jats:sc>\u2010alanine stretches were analyzed. As the full\u2010length protein displayed a strong tendency to aggregate into amorphous deposits, soluble N\u2010terminal fragments were also studied. Expansion of the poly\u2010<jats:sc>l<\/jats:sc>\u2010alanine sequence to the maximal length observed in OPMD patients led to an increase of \u03b1\u2010helical structure. Upon prolonged incubation the protein was found in fibrils that showed all characteristics of amyloid\u2010like fibers. The lag\u2010phase of fibril formation could be reduced by seeding. Structural analysis of the fibrils indicated antiparallel \u03b2\u2010sheets.<\/jats:p>","DOI":"10.1110\/ps.03214703","type":"journal-article","created":{"date-parts":[[2003,11,19]],"date-time":"2003-11-19T18:42:47Z","timestamp":1069267367000},"page":"2685-2692","source":"Crossref","is-referenced-by-count":52,"title":["Trinucleotide expansions leading to an extended poly\u2010<scp>l<\/scp>\u2010alanine segment in the poly (A) binding protein PABPN1 cause fibril 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