Focusing on components of the neuromuscular circuit which are affected in CMT and autosomal-dominant centronuclear myopathy [CNM, a second disease associated with a distinct set of DNM2 mutations (22)], ablation of DNM2 specifically in mouse skeletal muscle resulted in muscle fibre atrophy and loss, altered neuromuscular junctions, degenerating nerve fibres, accumulation of lipid droplets and mitochondrial abnormalities (23). This evidence concerns the gene DNM2 and centronuclear myopathy.