Posted on 13 August 2020
A paper published in the Journal of Cell Biology describes a new therapy that could be developed to prevent age-associated pulmonary fibrosis, a potentially lethal disease in which the lungs become increasingly scarred over time. It is associated with certain genetic defects and advanced age, and there is currently no cure.
Lung tissue of patients with pulmonary fibrosis does not regenerate because the cells involved in lung generation have damaged telomeres, the ends of the chromosomes. The new study describes a gene therapy that activates the enzyme that repairs telomeres.
“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 ageing,” says Maria Blasco, principal investigator of the study.
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 Blasco, this therapy was highly effective in animal models and no side effects were observed.
Telomeres are structures that protect the ends of chromosomes from damage. Part of the telomere is lost each time a cell divides, meaning that they become progressively shorter with age. Eventually, the cell is no longer able to divide at all. It appears that this telomere degradation and loss of replication is linked to pulmonary fibrosis, particularly when it occurs in alveolar cells called type II pneumocytes.
“We have observed a very clear relation between telomere status in type II pneumocytes, pulmonary surfactant production and fibrosis development in animals,” Pérez-Gil adds. “Here we address the effects on telomeres at the molecular level, biological and physical changes in cells and tissues, and the consequences for the health of the animal, the whole organism.”, indicates Sergio Piñeiro, first author of the study.
The researchers had previously shown that their therapy could reverse fibrosis in mice lacking telomerase entirely. However, this was not an ideal model of human disease, because most cases in humans are not caused by any one specific mutation. This study, on the other hand, shows that the therapy works in mice that only underwent physiological ageing – something that is common to almost all human cases.
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