Featured Research

from universities, journals, and other organizations

Breakthrough in understanding hereditary emphysema

Date:
January 24, 2011
Source:
Royal College of Surgeons in Ireland (RCSI)
Summary:
Researchers in Ireland have made a breakthrough in understanding the mechanisms behind the most severe form of hereditary emphysema and how protein treatments can improve the condition. The findings of this study may also lead to new treatments for patients with smoker’s emphysema.

Researchers from the Royal College of Surgeons in Ireland (RCSI) and Beaumont Hospital have made a breakthrough in understanding the mechanisms behind the most severe form of hereditary emphysema and how protein treatments can improve the condition. The findings of this study may also lead to new treatments for patients with smoker's emphysema.

Related Articles


The inherited condition, known as Alpha-1 Antitrypsin Deficiency (Alpha-1), results in the most severe form of hereditary emphysema. Alpha-1 Antitrypsin is an important protein produced by the liver, from where it is released into the bloodstream and travels to the lungs to protect the lungs from disease.

In patients with the Alpha-1 condition, excessive amounts of white blood cells (neutrophils) enter the lungs and cause inflammation and chronic lung disease. Until now the cause of this was not fully understood but the researchers involved in this study have now uncovered the mechanisms behind the influx of white blood cells which causes the inflammation.

The condition is more common in Ireland than in most other countries with severe deficiency occurring in 1 in 2,000 of the population and 1 in 24 people carry the gene for the disease. After cystic fibrosis, Alpha-1 is the most common fatal inherited lung condition in Ireland and in its most severe form is estimated to affect more than 2,000 people nationally. The less severe form may affect as many as 300,000 individuals in Ireland.

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 white blood cells entering the lungs, leading to the development of lung disease. Our research also reveals how a treatment known as augmentation therapy, where the natural Alpha-1 protein is given intravenously, leads to a decrease in the white blood cells going into the lungs thereby decreasing inflammation. This research gives new hope for a better quality of life for sufferers of this chronic condition."

The research may also be applied to those with smoker's emphysema. Smokers render themselves deficient in the alpha-1 protein as chemicals in cigarette smoke destroys this important protein which protects their lungs.

"We are now exploring the possibility of treating smoker's emphysema with the Alpha-1 protein" Professor McElvaney concluded.

Mr. John Walsh, President of the Alpha-1 Foundation, the US patient advocate group which provided research funding for this project, commented: "Typically Alpha-1 patients present with early onset emphysema somewhere between 35 and 45 years old. The impact on the individual who develops disease is significant and, according to medical literature in the US, the average lifespan of an Alpha-1 is 54 years. The research being carried out by Prof McElvaney and his team at RCSI Beaumont has really advanced the understanding of Alpha-1."

In 2004, the Alpha One Foundation initiated the first national screening programme for Alpha-1 in the world with funding from the Department of Health and Children. So far this programme has tested over 5,000 individuals and found over 25% are at risk.

The joint lead authors on the study are Dr David Bergin and Dr Emer Reeves from the Respiratory Research Division of RCSI's Department of Medicine based in the Education and Research Centre at Beaumont Hospital in Dublin who worked with a team at the Centre and also collaborated with researchers at Dublin City University.

The study was published in the Journal of Clinical Investigation in December 2010.

The research has additionally been recognised as being in the top 2% of published articles in biology and medicine by the distinguished Faculty of 1000. In their assessment, this study is a major contribution towards understanding the mechanism underlying excessive white blood cell (neutrophil) migration to the lungs of patients with hereditary alpha-1-antitrypsin deficiency.

The research was also supported by the Medical Research Charities Group/ Health Research Board, the Programme for Research in Third-Level Institutions (PRTLI) administered by the Higher Education Authority, the Alpha One Foundation (Ireland), Alpha-1 Foundation (USA) and the Department of Health & Children.


Story Source:

The above story is based on materials provided by Royal College of Surgeons in Ireland (RCSI). Note: Materials may be edited for content and length.


Journal Reference:

  1. David A. Bergin, Emer P. Reeves, Paula Meleady, Michael Henry, Oliver J. McElvaney, Tomαs P. Carroll, Claire Condron, Sanjay H. Chotirmall, Martin Clynes, Shane J. O’Neill, Noel G. McElvaney. α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8. Journal of Clinical Investigation, 2010; 120 (12): 4236 DOI: 10.1172/JCI41196

Cite This Page:

Royal College of Surgeons in Ireland (RCSI). "Breakthrough in understanding hereditary emphysema." ScienceDaily. ScienceDaily, 24 January 2011. <www.sciencedaily.com/releases/2011/01/110124081555.htm>.
Royal College of Surgeons in Ireland (RCSI). (2011, January 24). Breakthrough in understanding hereditary emphysema. ScienceDaily. Retrieved October 31, 2014 from www.sciencedaily.com/releases/2011/01/110124081555.htm
Royal College of Surgeons in Ireland (RCSI). "Breakthrough in understanding hereditary emphysema." ScienceDaily. www.sciencedaily.com/releases/2011/01/110124081555.htm (accessed October 31, 2014).

Share This



More Health & Medicine News

Friday, October 31, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

U.S. Ebola Response Measures Demonstrated

U.S. Ebola Response Measures Demonstrated

AP (Oct. 31, 2014) — Officials in the Washington area showed off Ebola response measures being taken at Dulles International Airport and the National Institutes of Health. (Oct. 31) Video provided by AP
Powered by NewsLook.com
Fauci Says Ebola Risk in US "essentially Zero"

Fauci Says Ebola Risk in US "essentially Zero"

AP (Oct. 30, 2014) — NIAID Director Anthony Fauci said the risk of Ebola becoming an epidemic in the U.S. is essentially zero Thursday at the Washington Ideas Forum. He also said an Ebola vaccine will be tested in West Africa in the next few months. (Oct. 30) Video provided by AP
Powered by NewsLook.com
Nurse Defies Ebola Quarantine With Bike Ride

Nurse Defies Ebola Quarantine With Bike Ride

AP (Oct. 30, 2014) — A nurse who vowed to defy Maine's voluntary quarantine for health care workers who treated Ebola patients followed through on her promise Thursday, leaving her home for an hour-long bike ride. (Oct. 30) Video provided by AP
Powered by NewsLook.com
Pot-Infused Edibles Raise Concerns in Colorado

Pot-Infused Edibles Raise Concerns in Colorado

AFP (Oct. 30, 2014) — Colorado may have legalized marijuana for recreational use, but the debate around the decision still continues, with a recent - failed - attempt to ban cannabis-infused edibles. Duration: 01:53 Video provided by AFP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:

Strange & Offbeat Stories

 

Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins