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Irish Study Reveals New Therapy for Hereditary Emphysema

Researchers from Ireland may have uncovered a new therapy for people who suffer from hereditary emphysema. That is according to a recent article published in the January 2014 issue of the journal of Science Translational Medicine, “The Circulating Proteinase Inhibitor α-1 Antitrypsin Regulates Neutrophil Degranulation and Autoimmunity.”

The study examined the ability of a serum α-1 antitrypsin (AAT) – a protein produced by the liver which, when released into the bloodstream, travels to the lungs to protect lung tissue from disease – to control tumor necrosis factor–α (TNF-α) – a contributor to many of the problems associated with autoimmune diseases. In addition, they assessed whether AAT deficiency (AATD), a hereditary disorder that leads to the most severe form of hereditary emphysema, is a TNF-α–related disease.

What they found is that AAT plays an important role in controlling inflammation from white blood cells.

"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,” said Professor Gerry McElvaney of the Royal College of Surgeons in Ireland. “This leads to an autoimmune process in the body that mistakenly recognizes these proteins as foreign and activates its own white blood cells to produce harmful oxidants."

These findings suggest that Alpha-1 is not purely a lung and liver disorder, but much more systemic, and treatments for Alpha-1 may also be used for other autoimmune disorders.

"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," said McElvaney said. “[In addition], the data would suggest we can decrease the progression of emphysema by using this therapy.”

Researchers are now working on a new treatment for Alpha-1 which involves taking purified Alpha-1 protein from the bloodstream of a person without a deficiency in AAT and giving it intravenously to people with a deficiency. The result is a decrease in the abnormal protein release, which alleviates the disease-associated autoimmunity.

Click Here to Access the Full Story from the journal of Science Translational Medicine.