Featured Research

from universities, journals, and other organizations

Cystic fibrosis: Additional immune dysfunction discovered

Date:
September 4, 2014
Source:
Helmholtz Zentrum Muenchen - German Research Centre for Environmental Health
Summary:
Cystic fibrosis (CF) is a frequent genetic disease affecting the lung and the gastrointestinal tract. Scientists have now shown that many of the adult patients with CF in addition lack a cell surface molecule, which is important for immune defense.

Cystic fibrosis (CF) is a frequent genetic disease affecting the lung and the gastrointestinal tract. Scientists from the Helmholtz Zentrum München now have shown that many of the adult patients with CF in addition lack a cell surface molecule, which is important for immune defence. The results have been published recently in the Journal of Molecular Medicine.

Related Articles


Cystic fibrosis (mucoviscidosis) is due to a mutation of an ion channel which leads to highly viscous mucus and to dysfunction of the lung and the gastrointestinal organs. Since these patients frequently suffer from chronic infections, Dr. Thomas Hofer and Professor Dr. Loems Ziegler-Heitbrock from the Comprehensive Pneumology Center (CPC) at Helmholtz Zentrum München -- together with colleagues at the Klinikum der Universität München and the University of Leicester, UK -- investigated, whether these patients might have an additional immune defect. The scientists found that the immunological cell surface molecule HLA-DQ is reduced or absent in many of these patients.

Defect is seen in all relevant leukocyte populations

HLA-DQ belongs to the MHC class II molecules, which can present crucial parts of invading microbes to immune cells such that the latter are activated leading to specific elimination of the pathogens. The class II molecules are strongly expressed on primary immune cells such as monocytes, macrophages and so-called dendritic cells. The study showed that HLA-DQ is reduced or absent in all these cell types in the blood and in the lung. Hence, all of the relevant antigen-presenting cells of the immune system are affected.

First insight into the molecular mechanism

In order to uncover the molecular mechanism behind this defect, the team studied the different steps in the molecular regulation In patients with a defect of HLA-DQ, the inflammatory messenger interferon-gamma was unable to induce the transcription factor CIITA along with a failure to increase HLA-DQ. What remains unclear is the cause of the deficient interferon response and the contribution of defective HLA-DQ to the course of the disease. In further studies the scientists aim to develop a rapid test system for the immune dysfunction that may be of great importance for diagnosis and treatment of cystic fibrosis.


Story Source:

The above story is based on materials provided by Helmholtz Zentrum Muenchen - German Research Centre for Environmental Health. Note: Materials may be edited for content and length.


Journal Reference:

  1. Thomas P. Hofer, Marion Frankenberger, Irene Heimbeck, Dorothe Burggraf, Matthias Wjst, Adam K. A. Wright, Maria Kerscher, Susanne Nährig, Rudolf M. Huber, Rainald Fischer, Loems Ziegler- Heitbrock. Decreased expression of HLA-DQ and HLA-DR on cells of the monocytic lineage in cystic fibrosis. Journal of Molecular Medicine, 2014; DOI: 10.1007/s00109-014-1200-z

Cite This Page:

Helmholtz Zentrum Muenchen - German Research Centre for Environmental Health. "Cystic fibrosis: Additional immune dysfunction discovered." ScienceDaily. ScienceDaily, 4 September 2014. <www.sciencedaily.com/releases/2014/09/140904084508.htm>.
Helmholtz Zentrum Muenchen - German Research Centre for Environmental Health. (2014, September 4). Cystic fibrosis: Additional immune dysfunction discovered. ScienceDaily. Retrieved February 26, 2015 from www.sciencedaily.com/releases/2014/09/140904084508.htm
Helmholtz Zentrum Muenchen - German Research Centre for Environmental Health. "Cystic fibrosis: Additional immune dysfunction discovered." ScienceDaily. www.sciencedaily.com/releases/2014/09/140904084508.htm (accessed February 26, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Thursday, February 26, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Sleeping Too Much Or Too Little Might Increase Stroke Risk

Sleeping Too Much Or Too Little Might Increase Stroke Risk

Newsy (Feb. 26, 2015) — People who sleep more than eight hours per night are 45 percent more likely to have a stroke, according to a University of Cambridge study. Video provided by Newsy
Powered by NewsLook.com
London Show Dissects History of Forensic Science

London Show Dissects History of Forensic Science

AFP (Feb. 25, 2015) — Forensic science, which has fascinated generations with its unravelling of gruesome crime mysteries, is being put under the microscope in an exhibition of real criminal investigations in London. Duration: 00:53 Video provided by AFP
Powered by NewsLook.com
Michigan Couple Celebrates Identical Triplets

Michigan Couple Celebrates Identical Triplets

AP (Feb. 25, 2015) — A suburban Detroit couple who have two older children are adjusting to life after becoming parents to identical triplets _ a multiple birth a doctor calls rare. (Feb. 25) Video provided by AP
Powered by NewsLook.com
Mayor Says District of Columbia to Go Ahead With Pot Legalization

Mayor Says District of Columbia to Go Ahead With Pot Legalization

Reuters - News Video Online (Feb. 25, 2015) — Washington&apos;s mayor says the District of Columbia will move forward with marijuana legalization, despite pushback from Congress. Rough Cut (no reporter narration). Video provided by Reuters
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