In this study, we evaluate if ASO‐mediated reduction of VCP ameliorates VCP‐related proteinopathy phenotypes in human induced pluripotent stem cells‐derived skeletal muscle progenitor cells (hiPSC‐SMPCs) with the most prevalent VCP R155H mutation and in the humanised VCP A232E mice.2, 34. Here, VCP is linked to proteostasis deficiencies.