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Ernesto Valdes MD, Guest Editor
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In Vitro Surfactant Protein B Deficiency Inhibits Lamellar Body Formation. Foster, Cherie D., Respiratory Cell & Molecular Biology (Aug 2003) 29;No 2; 259-266.
Surfactant protein B (SP–B) is essential for normal pulmonary surfactant activity and lamellar body genesis in type 2 cells. The lamellar body is involved in the synthesis, organization and secretion of pulmonary surfactant from the type 2 cells. However, the role of SP-B in lamellar body genesis is poorly understood. Disrupted lamellar body genesis is a prominent feature of SP-B deficiency.
We developed an adenovirus vector expressing antisense SP-B as an alternative in vitro model of SP-B deficiency. Antisense SP-B expression resulted in reliable in vitro reproduction of many features of SP-B Deficiency, including absent mature SP-B and decreased lamellar bodies and SP-C.
Comment: Genetically we are getting closer to the cure
instead of just treating symptoms with medications. E.V.
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