BSG and sarcoglycanopathy: Because of the lack of phenotype in the H77C α-SG mouse model and with respect to the need of an adequate model for evaluating pharmacological strategies able to rescue missense mutations leading to sarcoglycanopathies, we decided to create a new KI model that recapitulates a human missense mutation (T151R, Threonine to Arginine at position 151) in the β-SG protein.