Here, we uncover regulatory pathways of starvation‐induced autophagy by the RNA‐binding proteins, heterogeneous nuclear ribonucleoprotein Q (HNRNPQ) and poly(A) binding protein nuclear 1 (PABPN1), and showed that these pathways are perturbed by oculopharyngeal muscular dystrophy (OPMD) mutations. Here, PABPN1 is linked to oculopharyngeal muscular dystrophy.