BIN1 and myopathy: As skipping of the muscle-specific exon 11 of BIN1 causes ARCNM with highly progressive myopathy [40], a mouse model with deletion of Bin1 in-frame exon 11 (Bin1ex11−/−) was generated, causing an isoform switching from the muscle-specific isoform 8 (with exon 11) to the ubiquitous isoform 9 (without exon 11) in skeletal muscle [131].