Pulmonary surface-active materials in the Chediak-Higashi syndrome.

Beige mice express the Chediak-Higashi syndrome. Large inclusions, identified as abnormal lysosomes, are found in many cells. The inclusions in type II alveolar epithelial cells are enlarged lamellar bodies and they are associated with an increase in total lung surface-active material and phospholipid. Comparison of recovery of phospholipid in surface-active materials from beige and black (normal) mice indicates that in the beige mice there is an increase in total phospholipid and disaturated phosphatidylcholines in whole lung and in surface-active materials in residual lung after lavage. Hosphatidycholine and phosphatidylglycerol are increased as percentages of total lung phospholipid. Calculated alveolar surface coverage of surface-active materials isolated from residual beige lungs is greater than three times that of normal lungs. Surface-active materials recovered from beige mice are qualitatively similar in phospholipid composition and in surface activity to materials recovered from normal mice. The quantity of surface-active material phospholipid recovered in the lavage of beige mouse lungs was normal. The basis for the abnormal accumulation of lamellar body lipids is not known.