PABPN1 and oculopharyngeal muscular dystrophy: Decreased levels of mitochondrial proteins as well as poly(A) tail shortening was also found in another OPMD mouse model that closely reproduces Pabpn1 mutation in OPMD patients, i.e. Pabpn1-17ala/Pabpn1 at the endogenous Pabpn1 locus [24].