Surfactant protein B deficiency as a prototype
β Scribed by George B. Mallory
- Publisher
- John Wiley and Sons
- Year
- 2004
- Tongue
- English
- Weight
- 33 KB
- Volume
- 37
- Category
- Article
- ISSN
- 8755-6863
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We report on the DNA-based prenatal diagnosis of congenital pulmonary alveolar proteinosis in a family in which alveolar proteinosis was associated with surfactant protein B (SP-B) deficiency. The parents had lost an eight-week-old female child due to this fatal disorder. The affected child was homo
Pulmonary surfactant is a mixture of phospholipids and surfactant-associated proteins made by alveolar type II cells that is necessary for normal lung function. Surfactant secretion and reuptake by type II cells are regulated in part by interaction of surfactant protein-A (SP-A) with a specific rece