The Ubiquitin-Proteosome System: A Critical Regulator in Pulmonary Fibrosis

Introduction

Idiopathic pulmonary fibrosis (IPF)  is a chronic and irreversible disease of the lungs that presents in patients as ongoing cough and dyspnea (uncomfortable breathing) for a prolonged period without other underlying lung disease, and has been estimated to affect over 3 million people worldwide^1,2. This progressive disease is characterized by accumulating scarring of the lungs that diminishes quality of life, and has a 3-5 year survival time from diagnosis when untreated³.  Currently, nintedanib and pirfenidone (anti-fibrotic drugs) are the only approved treatments for IPF, and while they may extend survival by a few years with early intervention, they will not reverse the scarring and damage to the lungs already present⁴.  The molecular mechanisms that regulate IPF are not well defined but are presumed to be triggered by repetitive injury to the lungs and correlate with the most considerable risk factor for IPF which is aging. At the cellular level, mechanisms that affect cell aging and senescence include processes like telomere attrition and DNA damage, protein recycling and degradation pathways, and mitochondrial dysfunction^5,6.  Interestingly, several studies have linked the ubiquitin-proteasome system to IPF, and this newsletter will delve into why ubiquitination has emerged as a critical regulator of this disease.

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