Structure and function of the respiratory system of the dystrophic hamster

The BIO 14.6 dystrophic hamster has been used extensively over the past 30 years as an animal model in which to study the mechanisms responsible for the development of cardiomyopathy and skeletal muscle dysfunction associated with muscular dystrophy. More recently, structural and functional aspects of the respiratory system of this animal model have been investigated. This review summarizes our current knowledge of ventilation, lung morphometry and mechanics, the structure and function of the diaphragm, tracheal and pulmonary vascular smooth muscle, and pulmonary macrophages in the BIO 14.6 dystrophic hamster. We conclude that many aspects of the structure and function of the respiratory system of this hamster warrant further investigation, including the development of alveolar hypoventilation, the causes of pulmonary vascular hyporeactivity, and the potential contribution of abnormal pulmonary macrophages to the pathogenesis of life-threatening respiratory disease in muscular dystrophy.