Surfactant Protein A - From Genes to Human Lung Diseases

Title: Surfactant Protein A - From Genes to Human Lung Diseases

Volume: 13 Issue: 27

Author(s): S. Heinrich, D. Hartl and M. Griese

Affiliation:

Keywords: surfactant protein A, innate immunity, polymorphisms, lung diseases

Abstract: Surfactant associated protein-A (SP-A) is the most abundant pulmonary surfactant protein and belongs to the family of innate host defense proteins termed collectins. Besides pulmonary host defense, SP-A is also involved in the formation of pulmonary surfactant, as it is essential for the structure of tubular myelin. The human SP-A gene locus includes two functional genes, SFTPA1 and SFTPA2 which are expressed independently, and a pseudo gene. The largest amount of SP-A1 proteins assemble to larger molecular complexes, whereas SP-A2 forms mainly dimers and trimers. SPA polymorphisms play a role in respiratory distress syndrome, allergic bronchopulmonary aspergillosis and idiopathic pulmonary fibrosis. The levels of SP-A are decreased in the lungs of patients with cystic fibrosis, respiratory distress syndrome and further chronic lung diseases. Future areas for clinical research include disease specific SP-A expression pattern and their functional consequences, the differential roles of SP-A1 and SP-A2 in human lung diseases, and therapeutical approaches to correct altered SP-A levels.

Cite this article as:

Heinrich S., Hartl D. and Griese M., Surfactant Protein A - From Genes to Human Lung Diseases, Current Medicinal Chemistry 2006; 13 (27) . https://dx.doi.org/10.2174/092986706778773112