Collagen VI Deficiency Results in Structural Abnormalities in the Mouse Lung

Collagen VI (COL6) is known for its role in a spectrum of congenital muscular dystrophies, which are often accompanied by respiratory dysfunction. However, little is known regarding the function of COL6 in the lung. We confirmed the presence of COL6 throughout the basement membrane region of mouse lung tissue. Lung structure and organization were studied in a previously described Col6a1−/− mouse, which does not produce detectable COL6 in the lung. The Col6a1−/− mouse displayed histopathologic alveolar and airway abnormalities. The airspaces of Col6a1−/− lungs appeared simplified, with larger (29%; P