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Guttentag, S.H. et al.

Guttentag, Akhtar, Tao, Atochina, Rusiniak, Swank, Bates,

Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome.

Hermansky-Pudlak syndrome ( HPS) in humans represents a family of disorders of lysosome-related organelle biogenesis associated with severe, progressive pulmonary disease. Human case reports and a mouse model of HPS, the pale ear/pearl mouse ( ep/pe), exhibit giant lamellar bodies (GLB) in type II alveolar epithelial cells. We examined surfactant proteins and phospholipid from ep/pe mice to elucidate the process of GLB formation. The 2.8-fold enrichment of tissue phospholipids in ep/pe mice resulted from accumulation from birth through adulthood. Tissue surfactant protein (SP)-B and -C were increased in adult ep/pe mice compared with wild-type mice (WT), whereas SP-A and -D were not different. Large aggregate surfactant (LA) from adult ep/pe mice had decreased phospholipid, SP-B, and SP-C, with no differences in SP-A and -D compared with WT. Although LA from ep/pe animals exhibited an increased total protein-to-total phospholipid ratio compared with WT, surface tension was not compromised. Phospholipid secretion from isolated type II cells showed that basal and stimulated secretion from ep/pe cells were approximately 50% of WT cells. Together, our data indicate that GLB formation is not associated with abnormal trafficking or recycling of surfactant material. Instead, impaired secretion is an important component of GLB formation in ep/pe mice.[1]

References

Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome. Guttentag, S.H., Akhtar, A., Tao, J.Q., Atochina, E., Rusiniak, M.E., Swank, R.T., Bates, S.R. Am. J. Respir. Cell Mol. Biol. (2005) [Pubmed]

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