RYR1 and glycogen storage disease VI: This model recapitulates the main features observed in “Dusty core Disease” patients, a subgroup of Central Core Disease with exclusive RyR1 reduction, and due to a better homogeneity between the mice (same age, same sex, ...) than between the patients (different severity, different age at the biopsy, different age at onset of the disease, ...) and to the higher number of sample available, this mouse model has allowed to point to modifications that could shed a new light on the pathophysiological mechanisms, only partially known, and provide new clues for therapeutic development.