Evidence of defective surfactant biosynthesis in patients with Idiopathic Pulmonary Fibrosis (IPF)

Rationale: Idiopathic pulmonary fibrosis (IPF) is one of the most common forms of interstitial lung disease, however, the aetiology of IPF is poorly understood. An increasingly favoured working hypothesis suggests that epithelial injury is the primary triggering event. In this regard, abnormalities in expression and processing of the hydrophobic surfactant proteins SP-B and SP-C are suggested to contribute to the pathogenesis of IPF. Recently, gene mutations of SP-C have been found to result in misfolding and accumulation of the proprotein in ubiquitin-rich aggregates which are resistant to degradation thereby i…