PABPN1 and oculopharyngeal muscular dystrophy: Specific expression of alanine-expanded mammalian PABPN1 in Drosophila muscles reproduces the main features of OPMD: progressive muscle weakness and degeneration resulting in Drosophila wing position defects, and accumulation of mutant PABPN1 in nuclear aggregates in affected muscles [31,40].