The Role of Mitochondrial Dysfunction in Idiopathic Pulmonary Fibrosis: New Perspectives for a Challenging Disease

Simple Summary Idiopathic pulmonary fibrosis (IPF) presents a significant challenge. Despite extensive research, our understanding of its underlying causes remains limited. One intriguing hypothesis proposes that disruptions in mitochondrial functionality might play a role. Mitochondrial dysfunction is a recognized feature of the normal aging process, and parallels can be drawn to IPF, particularly within type 2 alveolar cells. This comprehensive review aims to unravel the various pathways of mitochondrial dysfunction observed in IPF. Furthermore, we explore potential therapeutic interventions designed to address this complex issue.Abstract Mitochondrial biology has always been a relevant field in chronic diseases such as fibrosis or cancer in different organs of the human body, not to mention the strong association between mitochondrial dysfunction and aging. With the development of new technologies and the emergence of new methodologies in the last few years, the role of mitochondria in pulmonary chronic diseases such as idiopathic pulmonary fibrosis (IPF) has taken an important position in the field. With this review, we will highlight the latest advances in mitochondrial research on pulmonary fibrosis, focusing on the role of the mitochondria in the aging lung, new proposals for mechanisms that support mitochondrial dysfunction as an important cause for IPF, mitochondrial dysfunction in different cell populations of the lung, and new proposals for treatment of the disease.

Automated summary

This article provides an overview of the various mechanisms of mitochondrial dysfunction in idiopathic pulmonary fibrosis (IPF) and explores potential therapeutic interventions.