Of the many ailments and diseases accompanying the aging process, not one of the least affects our lungs. Idiopathic Pulmonary Fibrosis (IPF) is a disease characterized by scarring in the lungs. The scar tissue that comes with this malady destroys normal lung tissues and their ability to oxygenate the blood. The median survival-time is between 2 to 3 years.

As with many age-related diseases, the onset of IPF is linked with telomere attrition and cellular senescence. Yet the critical role of telomeres in proper lung-functioning becomes even more apparent when we realize that short telomeres are a component of a variety of lung diseases. 

However, here we will focus on the role of telomere attrition in IPF. We will then look towards the horizon, for there is hope. Although as of now, there is no sanctioned and certain cure for IPF in humans, the experimentally proven potential of gene therapy for this malady is pointing towards a possible future remedy.

 As telomeres shorten, cells’ ability to carry out their proper functions are stifled. Weighty evidence suggests the attrition of telomeres and their proteomes is a major component to the onset of this disease. The proteomes of telomeres are a compound of more than 200 proteins that play an essential role in the protection and elongation of telomeres, as well as a key role in their ability to synthesize DNA.

In surveying the connection between telomere attrition and pulmonary fibrosis, Cronkite et al. found that one-fourth of cases are correlated with telomere lengths in the lower 10th percentile. 

Telomerase induction has been explored as a potential treatment. Povedano et al. has shown introducing adeno-associated virus (AAV) as vectors for gene therapy,  to activate telomerase in mice, resulted in the disappearance or improvement of fibrosis within eight weeks.  Significantly, remission coincided with a reversal in telomere shortening.

There is a steadily increasing literature on the promise of AAV in gene therapy. This benign virus that can be engineered to deliver DNA to target cells has been tested in cases of age-related diseases as diverse as Alzheimers and macular degeneration. 

BioViva and Integrated Health Systems are committed to tackling age-related diseases like Idiopathic Pulmonary Fibrosis with cutting-edge gene therapies. Let us come together and support this research so people may improve not only the quantity of their lives, but the quality of their health.

References and Suggested Reading

Hasancan Bilgili, Adam J. Białas, Paweł Górski, and Wojciech J. Piotrowski. Telomere Abnormalities in the Pathobiology of Idiopathic Pulmonary Fibrosis. J Clin Med. 2019 Aug; 8(8): 1232.Published online 2019 Aug 16. doi:10.3390/jcm8081232

Cronkhite J.T., Xing C., Raghu G., Chin K.M., Torres F., Rosenblatt R.L., Garcia C.K. Telomere shortening in familial and sporadic pulmonary fibrosis. Am. J. Respir. Crit. Care Med. 2008;178:729–737. doi: 10.1164/rccm.200804-550OC

Povedano J.M., Martinez P., Serrano R., Tejera Á., Gómez-López G., Bobadilla M., Flores J.M., Bosch F., Blasco M.A. Therapeutic effects of telomerase in mice with pulmonary fibrosis induced by damage to the lungs and short telomeres. Elife. 2018;7:e31299. doi: 10.7554/eLife.31299.

John Ryan
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