Ontology highlight
ABSTRACT:
SUBMITTER: Winter R
PROVIDER: S-EPMC3391145 | biostudies-literature | 2012 Jun
REPOSITORIES: biostudies-literature
Winter Reno R Kühn Uwe U Hause Gerd G Schwarz Elisabeth E
The Journal of biological chemistry 20120508 27
Oculopharyngeal muscular dystrophy is a late-onset disease caused by an elongation of a natural 10-alanine segment within the N-terminal domain of the nuclear poly(A)-binding protein 1 (PABPN1) to maximally 17 alanines. The disease is characterized by intranuclear deposits consisting primarily of PABPN1. In previous studies, we could show that the N-terminal domain of PABPN1 forms amyloid-like fibrils. Here, we analyze fibril formation of full-length PABPN1. Unexpectedly, fibril formation was in ...[more]