These findings using mouse models emphasize the need to study DNM2-mediated pathways in CNM pathogenesis and to understand the interlay between DNM2 and other disease genes causing ARCNM and XLCNM such as BIN1 and MTM1. The aim of this review is to further the understanding of CNM pathogenesis, by providing mechanistic insights into ADCNM (or DNM2-CNM), followed by discussion of DNM2 as a contributor to both ARCNM and XLCNM, and concluding with addressing DNM2 modulation as a potential therapeutic target for human disease. The gene discussed is DNM2; the disease is autosomal dominant centronuclear myopathy.