Telomerase-Directed Gene Therapy May Prove Effective Approach to Treating/Curing Idiopathic Pulmonary Fibrosis (IPF) Caused by Mutations or by Physioligcial Aging, New Results in Mouse Model Suggest

Idiopathic pulmonary fibrosis (IPF) is a potentially lethal disease for which there is currently no cure and that is associated with certain mutations or advanced age. The Telomeres and Telomerase Group at the Spanish National Cancer Research Centre (CNIO) had previously developed an effective therapy for mice with fibrosis caused by genetic defects. Now, these researchers have shown that the same therapy can be used successfully to treat mice with age-related fibrosis. "With respect to humans, our results indicate that it may be possible to devise a treatment to prevent the development of pulmonary fibrosis associated with aging," says Maria Blasco, PhD, Director of the CNIO and Head of the CNIO’s Telomeres and Telomerase Group, and principal investigator of the study that was published online on August 10, 2020 in The Journal of Cell Biology. The open-access article is titled “Telomerase Treatment Prevents Lung Profibrotic Pathologies Associated with Physiological Aging.” The treatment tested in mice is a gene therapy that activates the production of telomerase in the body. Telomerase is an enzyme that repairs the telomeres at the end of chromosomes. According to Dr. Blasco, this therapy was highly effective in animal models and no side effects were observed. Pulmonary fibrosis affects approximately 8,000 people in Spain and approximately 3 million individuals worldwide. Median lifespan from time of diagnosis is 2 to 4 years. In this disease, the lung tissue becomes stiff and scarred, and patients develop progressive shortness of breath. It is thought to be caused by a combination of genetic and environmental factors. Exposure to toxic substances plays an important role, but for the disease to manifest itself the patient must be of advanced age or have an underlying genetic condition.
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