Kathleen Glasser
The Connection Between Telomere Length and Idiopathic Pulmonary Fibrosis
Overview: IPF is a form of chronic pneumonia with an unknown cause. There is currently no diagnostic tool to identify IPF and the only known cure is lung transplantation. The goal of this literature review is to assess possible connections between telomere length and IPF as well as to develop possible diagnostic tools and treatments.Abstract: Idiopathic Pulmonary Fibrosis (IPF) is a specific form of chronic interstitial pneumonia characterized by the build up of fibrous tissue in the lungs.The disease affects over 5 million people worldwide and kills about 40,000 in the U.S. every year, about the same number of people who died from breast cancer in the U.S. in 2019 (Le Saux et al., 2013 and NIH, 2019). The disease causes a progressive decline in pulmonary function, an overall decline in health and well being, and ultimately leads to death with the median survival time being 2-3 years after diagnosis (Bilgili et al., 2019). While the cause of this disease is unknown, its association with telomere shortening and cellular senescence have indicated that telomere abnormalities play a major role in the pathobiology of IPF. A mutation in the TERT or TERC complex of telomerase is known to cause shortened telomeres and mutations in these genes have been identified in some IPF patients. However it is still unclear how shortened telomeres play a role in the progression of the disease. There are currently no diagnostic tools to identify IPF in individuals and the only known cure is lung transplantation. The goal of this literature review is to assess the possible connections between telomere length and IPF as well as to develop possible diagnostic tools and treatments for the disease.
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