Sarcoglycanopathies are rare autosomal recessive forms of the limb girdle muscular dystrophies family (LGMDR3-6). The main feature of these diseases is the progressive weakness of skeletal muscles of shoulder and pelvic girdle. The cause is the mutation of the sarcoglycan (SG) genes resulting in severe reduction or absence of the SG complex at the muscle membrane (sarcolemma). Notably, the most frequently reported genetic variants in LGMDR3 are missense mutations, resulting in misfolded proteins that are removed through the quality-control system even though potentially functional. Through the use of a small molecule, the C17 CFTR corrector, the human R98H-alpha-SG was efficiently rescued in a new mouse model for LGMDR3 in terms of reduction of the dystrophic phenotype and recovery of muscle force amelioration of the pathology and recovery of the force. To understand the suitability of C17 for clinical development, we performed ADME (absorption, distribution, metabolism, and excretion) studies. These analyses indicated that C17 CFTR corrector is absorbed, well-distributed to all major organs, and metabolized in the intestine into hydrophilic and hydrophobic forms. The elimination occurred through faeces (unmodified and modified C17) and urine (modified forms). Interestingly, HPLC analyses of treated muscle evidenced a quantifiable amount of C17 after 48 hours from the administration. This finding suggested testing a reduced dosing frequency regimen that led to recovery of tibialis anterior strength due to sarcolemma rescue of the mutated sarcoglycan and SG-complex. On the whole we evidenced the good pharmacological profile of C17 that has the potential to become a therapeutic strategy.