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Alpha-dystroglycan (αDG) is a highly glycosylated cell surface protein with a significant role in cell-to-extracellular matrix interactions in muscle. Abnormalities in the glycosylation of αDG is the pathogenic mechanism for the α-dystroglycanopathies, a group of congenital muscular dystrophies. Muscle biopsies were evaluated from three related female 6-week-old Labrador Retriever puppies with poor suckling, small stature compared to normal litter mates, bow-legged stance and markedly elevated creatine kinase activities (10,000-23,000 IU/L; reference 59-895 IU/L).