Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Newly discovered proteins associated with cystic fibrosis

06.11.2006
Researchers have found a highly unusual distribution of two proteins in the lungs and airways of people with cystic fibrosis, a discovery that could be a step in determining how the disease progresses. The proteins, first uncovered as a result of the human genome project, are thought to play a role in the body's immune system.

The discovery is preliminary, but intriguing: Finding out more about the proteins could help sort out the immune system's role in cystic fibrosis, a genetic disease that attacks the lungs and other organs and dramatically shortens life expectancy.

The study "Comparative expression of SPLUNC1, SPLUNC2 and LPLUNC1 in normal and diseased lungs," was carried out by Lynne Bingle of the University of Sheffield School of Clinical Dentistry, Sheffield, United Kingdom, and Colin Bingle, University of Sheffield Medical School, Sheffield. They will present the study at a meeting of The American Physiological Society, "Physiological Genomics and Proteomics of Lung Disease," on Nov. 3. The meeting takes place Nov. 2-5 in Fort Lauderdale.

"Our results show unique expression domains for (the proteins) within the airways and suggest that alterations in expression of these putative innate immune molecules may be associated with lung disease," the authors wrote.

Recently discovered proteins

"We've shown these proteins to be expressed in places like the upper airways, nose and mouth, where many bacteria and infectious agents are found," Bingle said. These tiny molecules are thought to be part of the first line of the body's defenses against infectious agents, Bingle said.

The human genome project localized the PLUNC (palate, lung and nasal epithelium clone) gene to chromosome 20. A genetic locus in this region directs the production of a family of at least 10 proteins. Some of the proteins are short, referred to as "SPLUNCs," while others are long, referred to as "LPLUNCs."

Researchers have found that these proteins locate in specific places in the bodies of healthy people. Because these proteins are found in different areas, researchers speculate that they may have slightly different functions and may fight different infectious agents.

Investigation with cystic fibrosis

People with cystic fibrosis have a faulty gene, which normally controls the movement of salt into and out of cells and this controls the movement of water, too, Bingle explained. In a patient with cystic fibrosis, there is too little salt and water on the outside of cells lining the airways. This means that the normally thin protective layer of mucus becomes thick and very difficult to move.

It is very difficult for the patient to cough up the thick mucus, so the airways get clogged. The trapped mucous becomes a haven for infectious agents, which leads to long-term infection, inflammation and scarring. Most patients will eventually need lung transplants in order to survive, she said.

In this study, the researchers compared the tissue of 21 cystic fibrosis patients -- all of whom had end stage disease and were scheduled for a transplant -- to healthy tissue. The healthy tissue was obtained from 10 patients undergoing surgical removal of a lung tumor. The researchers used the healthy portion of lung tissue which is usually excised along with the tumor.

The study looked at tissue samples from the lung's upper airway, just below the trachea, and from lower down in the airway, in the peripheral lung, where gas exchange takes place.

This lower region of the lung has small airways as well as the gas-exchange tissue. They used a staining technique to find SPLUNC1, SPLUNC2 and LPLUNC1.

In normal lungs:

SPLUNC1 is found predominantly in the upper airways, rarely in the smaller airways and is absent in the gas-exchange tissue of the peripheral lung.

LPLUNC1 is found in both the small airways and in the upper airways

SPLUNC2 is found in the mouth, but not in the lungs

The study found that the presence of SPLUNC1 is "massively increased in the small airways of the lungs of people with cystic fibrosis," Bingle said. "It is really difficult to find SPLUNC 1 in similar airways from the normal lung."

LPLUNC1 also increases significantly in the small airways of people with cystic fibrosis compared to normal tissue, Bingle noted. It is normal to have LPLUNC1 in this region, but people with cystic fibrosis have a much greater amount of it here.

The study found no differences in SPLUNC2 between the diseased and normal lungs. SPLUNC2 appears to be exclusively expressed in the mouth.

A step in the fight against cystic fibrosis

These findings could become a way to prevent cystic fibrosis related lung damage, most of which occurs because of the constant infections these people suffer, Bingle said. The body's immune reaction to the infection also damages the lungs, "so knowing how these immune reactions happen could help doctors prevent them or harness them to fight the infection before it becomes established," she said.

Christine Guilfoy | EurekAlert!
Further information:
http://www.the-aps.org

Further reports about: Bingle Infectious SPLUNC2 cystic cystic fibrosis fibrosis

More articles from Life Sciences:

nachricht Researchers uncover protein-based “cancer signature”
05.12.2016 | Universität Basel

nachricht The Nagoya Protocol Creates Disadvantages for Many Countries when Applied to Microorganisms
05.12.2016 | Leibniz-Institut DSMZ-Deutsche Sammlung von Mikroorganismen und Zellkulturen GmbH

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Shape matters when light meets atom

Mapping the interaction of a single atom with a single photon may inform design of quantum devices

Have you ever wondered how you see the world? Vision is about photons of light, which are packets of energy, interacting with the atoms or molecules in what...

Im Focus: Novel silicon etching technique crafts 3-D gradient refractive index micro-optics

A multi-institutional research collaboration has created a novel approach for fabricating three-dimensional micro-optics through the shape-defined formation of porous silicon (PSi), with broad impacts in integrated optoelectronics, imaging, and photovoltaics.

Working with colleagues at Stanford and The Dow Chemical Company, researchers at the University of Illinois at Urbana-Champaign fabricated 3-D birefringent...

Im Focus: Quantum Particles Form Droplets

In experiments with magnetic atoms conducted at extremely low temperatures, scientists have demonstrated a unique phase of matter: The atoms form a new type of quantum liquid or quantum droplet state. These so called quantum droplets may preserve their form in absence of external confinement because of quantum effects. The joint team of experimental physicists from Innsbruck and theoretical physicists from Hannover report on their findings in the journal Physical Review X.

“Our Quantum droplets are in the gas phase but they still drop like a rock,” explains experimental physicist Francesca Ferlaino when talking about the...

Im Focus: MADMAX: Max Planck Institute for Physics takes up axion research

The Max Planck Institute for Physics (MPP) is opening up a new research field. A workshop from November 21 - 22, 2016 will mark the start of activities for an innovative axion experiment. Axions are still only purely hypothetical particles. Their detection could solve two fundamental problems in particle physics: What dark matter consists of and why it has not yet been possible to directly observe a CP violation for the strong interaction.

The “MADMAX” project is the MPP’s commitment to axion research. Axions are so far only a theoretical prediction and are difficult to detect: on the one hand,...

Im Focus: Molecules change shape when wet

Broadband rotational spectroscopy unravels structural reshaping of isolated molecules in the gas phase to accommodate water

In two recent publications in the Journal of Chemical Physics and in the Journal of Physical Chemistry Letters, researchers around Melanie Schnell from the Max...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ICTM Conference 2017: Production technology for turbomachine manufacturing of the future

16.11.2016 | Event News

Innovation Day Laser Technology – Laser Additive Manufacturing

01.11.2016 | Event News

#IC2S2: When Social Science meets Computer Science - GESIS will host the IC2S2 conference 2017

14.10.2016 | Event News

 
Latest News

IHP presents the fastest silicon-based transistor in the world

05.12.2016 | Power and Electrical Engineering

InLight study: insights into chemical processes using light

05.12.2016 | Materials Sciences

High-precision magnetic field sensing

05.12.2016 | Power and Electrical Engineering

VideoLinks
B2B-VideoLinks
More VideoLinks >>>