Regulation and localization of the Telomerase- and the Shelterin-telosom-complex in sporadic Idiopathic Pulmonary Fibrosis

Introduction: Idiopathic pulmonary fibrosis (IPF) is an adult-onset, lethal, scarring lung disease of unknown etiology. Reports linking telomerase mutations to familial IPF suggest that reduced telomerase activity and defective telomere length maintenance are key factors in disease pathogenesis. Accelerated telomere shortening is also observed in sporadic IPF in the absence of gene mutations. We therefore aimed to analyze the expression of the Telomerase- [TERT,TERC,DKC1,NOP10,NHP2] and the Shelterin-telosom-complex [TERF1/-2,POT1,TIN2,PTOP,RAP1] in lungs from patients with sporadic IPF (n=30) and organ donors (…