Breakthrough in Understanding Hereditary Emphysema

Researchers from the Royal College of Surgeons in Ireland (RCSI) and Beaumont Hospital have made an important breakthrough in the understanding and treatment of hereditary emphysema.  Their research findings were published in this month’s edition of Science Translational Medicine, a prestigious journal that highlights medical advances resulting from scientific research, thus bridging the research-to-treatment gap. Their exciting findings show how the protein Alpha-1 Antitrypsin (AAT) plays an important role in controlling inflammation from white blood cells and its importance for good health.

The research found that Alpha-1 Antitrypsin (AAT) is an important protein produced by the liver which, when released into the bloodstream travels to the lungs to protect the  from disease. Patients deficient in AAT suffer from Alpha-1 Antitrypsin Deficiency (Alpha-1); a  that leads to the most severe form of hereditary emphysema.

Professor Gerry McElvaney, Professor of Medicine at RCSI and senior author on the study commented: “Our study is the first to reveal the mechanisms by which a lack of the Alpha-1 protein causes an increase in the release of white blood cell proteins into the blood stream. This leads to an autoimmune process in the body that mistakenly recognises these proteins as foreign and activates its own  to produce harmful oxidants”

“Our research also reveals how a treatment known as augmentation therapy, where Alpha-1 protein purified from blood, is given intravenously, leading to a decrease in the abnormal protein release thereby alleviating the disease associated autoimmunity. This research gives new hope for a better quality of life for sufferers of this chronic condition and may also be applied to other autoimmune associated diseases such as rheumatoid arthritis, Prof McElvaney continued.

Source: Medical Express 13/01/2014