RYR1 and myotonic dystrophy: Taken together, our data clearly indicate that the mis-regulated splicing events lead to the production of proteins with altered properties, and suggest that the deregulation of Ca2+ homeostasis in myotonic dystrophy could be determined by the failure to complete the developmental switch to a specific set of alternatively spliced genes, resulting in insufficient Ca2+ release from the ER/SR due to the reduction of functional RyR1 protein levels.