Surfactant protein B (SP-B) mRNA and protein are restricted to alveolar Type II and Clara
cells in the respiratory epithelium. In order to investigate the function of SP-B in these distinct …

Several human respiratory disorders have been linked to an abnormality of pulmonary
surfactant synthesis or turnover. Among those conditions, hereditary deficiency in the …

Pulmonary alveolar proteinosis (PAP) is a disorder that rapidly leads to respiratory failure,
because the alveolar spaces fill with a lipid-rich, proteinaceous material that impedes gas …

In both humans and mice, a deficiency of surfactant protein B (SP-B) is associated with a
decreased concentration of mature SP-C and accumulation of a larger SP-C peptide …

Surfactant protein B (SP-B) is required for function of newborn and adult lung, and partial
deficiency has been associated with susceptibility to lung injury. In the present study …

SFTPC (surfactant protein C) mutations resulting in SP-C deficiency causing ongoing
respiratory failure in the neonatal period represent a rare entity. We report a full-term female …

Surfactant replacement preparations containing either surfactant protein (SP)-B or SP-C
significantly improve lung function in surfactant-deficient infants, suggesting that these …

(CHEST 1997; 111: 129S) prostacyclin synthase (PGIS) is the final committed enzymatic
step in the pathway of prostacyclin (PGI2) production, occurring at a branch point where …

Surfactant Protein B Deficiency is a rare but lethal monogenetic, congenital lung disease of
the neonate that is unresponsive to any treatment except lung transplantation. Based on the …

Common causes of neonatal respiratory distress include meconium aspiration, pneumonia,
persistent pulmonary hypertension of the newborn, pneumothorax and cystic adenomatoid …