PABPN1 and oculopharyngeal muscular dystrophy: The high-scoring isoforms resembles prototypical prion-like proteins, containing two RNA-recognition motifs (RRMs) and a C-terminal PrLD (which is absent in the low-scoring isoform, and hnRNP A/B proteins were shown to co-aggregate with PABPN1 in a mammalian cell model of oculopharyngeal muscular dystrophy [57].