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Pathogenesis Of IPF

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Recommended: Role of genes in pathogenesis of IPF

It has been established that the genetic and environmental background play a major role in the development of IPF. Understanding the pathogenesis through genetic linkage analysis can provide an insight into the principal driver of lung scarring and honeycombing, both characteristics of IPF. Consistent with what Kropski et.al, (2013) put forward, there are four genes that cause familial IPF, the inherited variant of IPF, and control the pulmonary linked “surfactant protein C (encoded by SFTPC)” and the “surfactant protein A2 (encoded by SFTPA2)”. The surfactant protein C is known to be hydrophobic in nature and is crucial for normal lung operation and eventually survival of the human being. According to Kropski et.al, (2013), these four genes …show more content…

The authors also came up with the “multiple hit” model to explain and understand the pathogenesis of IPF. This model proposes that IPF is more a result of a combination of genetics and multiple environmental ‘hits’ that eventually leave the Type II alveolar epithelial cells susceptible to development of lung scarring and modification of the lung architecture. The Seibold et.al, 2011 study has theorized, as one of three possibilities, that the MUC5B mutation damages the mucosal host resistance mechanism causing disproportionate lung scarring and eventually leading to an advance of idiopathic interstitial pneumonia. According to the ‘multiple hit’ theory, the occurrence of interstitial pneumonia and GERD will eventually lead to scarring of the lungs and the subsequent outbreak of …show more content…

According to Marmai et.al, (2011), the EMT process is innately symbolized by a shrinkage in epithelial cell related genes like “E-cadherin” and a growth in mesenchymal cell related genes like “type I collagen (Col1) or α-smooth muscle actin (α-SMA)”. To prove that EMT transpires in an IPF lung, Marmai et.al, (2011) initially went through the two-step RT-PCR process, a sophisticated gene manifestation profiling system that uses laser capture to extract epithelial cells from IPF lung tissues. To add to this a thorough set of Immunohistochemical, Immunoblotting, Type II cell isolation & culturation tests were carried out. The epithelial cells captured by the two-step RT-PCR process demonstrated genes generally linked to the mesenchymal cell phenotype. Based on the multiple tests performed, the TGF-β prototype is the chief promoter of EMT linked to lung

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