PABPN1 and Alzheimer disease: Currently, ~30 genes of distal weakness (MYH7, HSPB8, FLNC, BAG3, VCP, DESMIN, DNAJB6, HNRNPA1, ACTN2, ACTA1, NOTCH2NLC, LRP12, GIPC1, CRYAB, MYOT, ZASP, TIA1, TTN, MATR3, SQSTM1, SQSTM1/TIA1, and PLIN4) and proximal weakness (COL6A2, CACNA1S, TNPO3, D4Z4, SMCHD1, HNRNPDL, PABPN1, and CAV3) are associated with the autosomal dominant (AD) form of RVM.