ERC Starting Grant · 2022
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 onset. Early clinical studies indicate that danazol modulates telomere length and prevents the progression of pulmonary fibrosis but it is poorly tolerated by patients, causing liver toxicity. Gene…
From the public funding record at EU CORDIS. Describes the funded project, not the reviews below.
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