Surfactant gene polymorphisms and interstitial lung diseases

• Type of Material: Article
• Authors: Panagiotis Pantelidis, National Heart and Lung Institute; Srihari Veeraraghavan, Emory University; Roland M. du Bois, National Heart and Lung Institute
• Language: English
• Date: 2001-11-29
• Publisher: BIOMED CENTRAL LTD
• Publication Version: Final Published Version
• Copyright Statement: © 2001, BioMed Central Ltd
• Final Published Version (URL): http://dx.doi.org/10.1186/rr163
• Title of Journal or Parent Work: RESPIRATORY RESEARCH
• Volume: 3
• Issue: 1
• Start Page: 14
• End Page: 14
• Keywords: NECROSIS-FACTOR-ALPHA; ACE GENE; HUMAN SP-A; INTERLEUKIN-1 RECEPTOR ANTAGONIST; interstitial lung disease; RESPIRATORY-DISTRESS SYNDROME; Respiratory System; MAJOR HISTOCOMPATIBILITY COMPLEX; polymorphism; Science & Technology; genetics; surfactant; CONVERTING ENZYME GENE; Life Sciences & Biomedicine; CONGENITAL ALVEOLAR PROTEINOSIS; SP-B DEFICIENCY; IDIOPATHIC PULMONARY FIBROSIS
• Research Categories: Health Sciences, Medicine and Surgery; Biology, Genetics

Abstract

Pulmonary surfactant is a complex mixture of phospholipids and proteins, which is present in the alveolar lining fluid and is essential for normal lung function. Alterations in surfactant composition have been reported in several interstitial lung diseases (ILDs). Furthermore, a mutation in the surfactant protein C gene that results in complete absence of the protein has been shown to be associated with familial ILD. The role of surfactant in lung disease is therefore drawing increasing attention following the elucidation of the genetic basis underlying its surface expression and the proof of surfactant abnormalities in ILD. © 2002 BioMed Central Ltd.