CORDIS Project
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This project aims to develop a human preclinical model to study idiopathic pulmonary fibrosis (IPF) by using patient-derived lung cells. It focuses on understanding telomere shortening in lung cells and testing targeted gene therapies to improve treatment outcomes and patient survival.
Idiopathic pulmonary fibrosis (IPF) is a lethal scarring lung disease often leading to death within 3 years of diagnosis.
New antifibrotic medications slow disease progression but they are poorly tolerated by patients.
Telomeres, protective structures or caps at the ends of chromosomes, governed by the enzyme telomerase are shortened in type 2 alveolar epithelial cells (AT2)s in sporadic IPF but particularly so in inherited mutations of telomere related genes causing severe disease with early on…
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