Featured Research

from universities, journals, and other organizations

Lost Tolerance Is A Grave Omenn

Date:
February 5, 2005
Source:
Journal Of Clinical Investigation
Summary:
Patients with Omenn syndrome have only a small number of T cells that escape thymic selection, infiltrate peripheral tissues, and cause autoimmune reactions. The origin of the autoreactive T cells that cause autoimmunity in Omenn syndrome was previously unclear.

Omenn syndrome is a rare, inherited, and often-fatal immune disease associated with defective T and B cell development. It is caused by mutations in recombinase activating genes RAG1 or RAG2, which hamper B and T cell generation. As a result, patients with Omenn syndrome have only a small number of T cells that escape thymic selection, infiltrate peripheral tissues, and cause autoimmune reactions. The origin of the autoreactive T cells that cause autoimmunity in Omenn syndrome was previously unclear.

In a new Journal of Clinical Investigation report appearing online on February 3 in advance of publication in the March 1 print edition, Raffaele Badolato and colleagues at the University of Brescia study autoimmune regulator element (AIRE) expression in thymuses from three deceased infants with mutations in RAG - two with Omenn syndrome and one with a related immunodeficiency. They find that these thymuses have greatly reduced AIRE levels compared to thymuses from healthy children. AIRE is expressed in the thymus and helps establish central tolerance and prevent organ-specific autoimmunity. In the presence of AIRE, peripheral self-antigens are presented and autoreactive T cells are eliminated whereas in the absence of AIRE, such clones survive and lead to self-reactivity.

The authors propose that AIRE deficiency in Omenn syndrome causes a lack of expression of tissue-specific antigens in the thymus, with consequent loss of central tolerance. These results provide insight into the development of autoimmunity in immunodeficient patients.


Story Source:

The above story is based on materials provided by Journal Of Clinical Investigation. Note: Materials may be edited for content and length.


Cite This Page:

Journal Of Clinical Investigation. "Lost Tolerance Is A Grave Omenn." ScienceDaily. ScienceDaily, 5 February 2005. <www.sciencedaily.com/releases/2005/02/050204214932.htm>.
Journal Of Clinical Investigation. (2005, February 5). Lost Tolerance Is A Grave Omenn. ScienceDaily. Retrieved September 1, 2014 from www.sciencedaily.com/releases/2005/02/050204214932.htm
Journal Of Clinical Investigation. "Lost Tolerance Is A Grave Omenn." ScienceDaily. www.sciencedaily.com/releases/2005/02/050204214932.htm (accessed September 1, 2014).

Share This




More Health & Medicine News

Monday, September 1, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Get on Your Bike! London Cycling Popularity Soars Despite Danger

Get on Your Bike! London Cycling Popularity Soars Despite Danger

AFP (Sep. 1, 2014) Wedged between buses, lorries and cars, cycling in London isn't for the faint hearted. Nevertheless the number of people choosing to bike in the British capital has doubled over the past 15 years. Duration: 02:27 Video provided by AFP
Powered by NewsLook.com
Can You Train Your Brain To Eat Healthy?

Can You Train Your Brain To Eat Healthy?

Newsy (Sep. 1, 2014) New research says if you condition yourself to eat healthy foods, eventually you'll crave them instead of junk food. Video provided by Newsy
Powered by NewsLook.com
We've Got Mites Living In Our Faces And So Do You

We've Got Mites Living In Our Faces And So Do You

Newsy (Aug. 30, 2014) A new study suggests 100 percent of adult humans (those over 18 years of age) have Demodex mites living in their faces. Video provided by Newsy
Powered by NewsLook.com
Liberia Continues Fight Against Ebola

Liberia Continues Fight Against Ebola

AFP (Aug. 30, 2014) Authorities in Liberia try to stem the spread of the Ebola epidemic by raising awareness and setting up sanitation units for people to wash their hands. Duration: 00:41 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:
from the past week

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