TY - JOUR T1 - Understanding the regulation of surfactant gene expression JF - European Respiratory Journal JO - Eur Respir J SP - 6 LP - 7 DO - 10.1183/09031936.03.00000103 VL - 22 IS - 1 AU - W. Jacot AU - J. Bousquet Y1 - 2003/07/01 UR - http://erj.ersjournals.com/content/22/1/6.abstract N2 - Among a number of biochemical and morphological criteria, alveolar type-II epithelial cells are defined by the synthesis of surfactant proteins. These molecules are implicated in numerous human diseases such as neonatal respiratory distress syndrome 1 and possibly acute respiratory distress syndrome (ARDS) 2, where a better understanding could lead to new therapeutic modalities via a targeted induction of surfactant synthesis. The synthesis of surfactant proteins (SPs) is under the control of a variety of potential developmental and hormonal regulators, however, two transcription factors, thyroid transcription factor (TTF)‐1 and hepatocyte nuclear factor (HNF)‐3, have a crucial role in mediating the expression of SPs 3. The promoter region of the SP‐B gene has been studied extensively 4. In the human SP‐B gene, a region located at the immediate 5′ flanking region of the basal promoter TATA box between 80–110 contains two cis-acting elements for TTF‐1 and HNF‐3 binding 4. These two elements are important for the specificity and activation of SPs gene expression in the lung. SP‐B is a 79-amino acid peptide critical to postnatal respiratory adaptation. SP‐B is the only surfactant-associated protein required for postnatal lung function and survival. Complete deficiency of SP‐B in mice and humans results in lethal neonatal respiratory distress syndrome and is characterised by a virtual absence of lung compliance, highly disorganised lamellar bodies, and greatly diminished levels of SP‐C mature … ER -