We report here the discovery of the first mutation in the canine sarcoglycan alpha subunit (SGCA) gene in young adult miniature dachshunds that leads to a form of LGMD with clinical signs that include subclinical myopathy with hyperCKemia, exercise intolerance, progressive weakness, variable dysphagia and pneumonia, and myoglobinuria. Here, SGCA is linked to limb-girdle muscular dystrophy.