To understand the particular function of BIN1 muscle-specific isoforms encompassing exon 11 in muscle development and maintenance, we constitutively deleted this in-frame exon to obtain Bin1ex11−/− mice expressing only ubiquitous BIN1 isoforms in skeletal muscle and mimicking BIN1 exon 11 skipping found in myotonic dystrophy, and in the highly progressive form of CNM (Fugier et al., 2011; Böhm et al., 2013). This evidence concerns the gene BIN1 and centronuclear myopathy.