Pulmonary fibrosis affects 8,000 people in Spain. Today there is no cure. However, now, the Telomere and Telomerase Group of the National Cancer Research Center (CNIO) has managed to cure this disease in mice, through gene therapy that treats it, for the first time, as an age-related disease.
And, how have they achieved it? Lengthening the telomeres of cells in damaged tissue.
Telomeres are the structures that line the end of chromosomes and are an important molecular indicator of health and longevity. Longer telomeres mean greater longevity and better health. For its part, telomerase is the enzyme that is capable of lengthening telomeres to confer additional life to the cell.
Only tumor cells can activate telomerase to make themselves immortal. But in the laboratory, the Telomeres and Telomerase Group, led by the director of the CNIO, Maria A. Blasco, has managed to activate telomerase in the cells of the lungs of pulmonary fibrosis patients, managing to reverse the disease; yes, in rodents.
This is how the authors describe it in their publication on January 30 in the eLife magazine. Given that telomere shortening is also an indicator of aging of the organism, Blasco emphasizes that “it is the first time that the treatment of pulmonary fibrosis has been approached as an age-related disease, seeking to rejuvenate the affected tissues”.
“The most relevant thing about our work is that it suggests a potentially viable and effective solution to a real clinical problem, pulmonary fibrosis, which so far has no treatment,” says Paula Martínez, co-author of the work.
“The only treatments for pulmonary fibrosis approved today do not have curative effects, since they attack a symptom of fibrosis and not the cause. Our therapy is based on correcting the molecular cause of pulmonary fibrosis in patients with short telomeres, introducing in cells in damaged lung tissue have the only enzyme capable of lengthening telomeres, telomerase. “
As detailed in a statement issued by the CNIO: “In pulmonary fibrosis, the lung tissue develops scars that cause a progressive loss of respiratory capacity. Environmental toxins play an important role in its origin, but it is known that it must There is also telomeric damage for the disease to appear. Pulmonary fibrosis patients have short telomeres whether the disease is hereditary – it runs in the family – or not. The most likely explanation is that when the telomeres become too short the Damage in the cell activates a ‘repair program’ that induces scar formation, and scar formation leads to fibrosis. “
/ National Center for Oncological Research (CNIO).
Reference: ‘ Therapeutic effects of telomerase in mice with pulmonary fibrosis induced by damage to the lungs and short telomeres’ . Maria Blasco, Juan Manuel Povedano, Paula Martinez, Rosa Serrano, Agueda Tejera, Gonzalo Gomez, Maria Bobadilla, Juana Maria Flores and Fatima Bosch (eLife 2018). DOI: 10.7554 / eLife.31299
