According to an article from news-medical.net, a group of researchers from the Telomere and Telomerase Group at the Spanish National Cancer Research Centre have been able to pin down the genetic link to idiopathic pulmonary fibrosis. With this new information a new gene therapy treatment has been developed and is showing signs of promise in early tests.
While pulmonary fibrosis can be caused by a variety of factors, idiopathic pulmonary fibrosis refers to fibrosis that does not have a clear cause. The disease is characterized by scarring of the lung tissues that progressively worsens. The average lifespan after diagnosis is only four years. Symptoms include a dry cough, progressively worsening shortness of breath, and fatigue. To learn more about this disease, click here.
The research concluded that the primary cause of idiopathic pulmonary fibrosis (IPF) was linked to damage to the telomeres, which protect the ends of chromosomes. Telomeres shorten naturally over time and causes aging in humans. Premature damage or shortening can lead to cell death and genetic damage. Researches propose the that the enyzme telomerase could be used to combat IPF and prevent the early genetic damage that causes the disease.
Currently, all treatments for IPF are primarily meant to be supportive by reducing or slowing the effects of symptoms and maintaining lung functionality. However, telomerase could provide a curative solution to IPF by reversing telomere damage. In order to test this possibility, the group developed a new animal model of IPF, as the previously used model did not take into the account the genetic cause. Two months of treatment with telomerase was enough to either cure or improve symptoms in the mouse model. They introduced the telomerase encoding gene to the diseased mice with the use of a viral vector.
