Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Recycler protein helps prevent disease

01.05.2009
Researchers identify protein recycling mechanism that helps protect from genetic disorders

Recycling is important not only on a global scale, but also at the cellular level, since key molecules tend to be available in limited numbers. This means a cell needs to have efficient recycling mechanisms.

Researchers at the European Molecular Biology Laboratory (EMBL) and Heidelberg University, Germany, have now uncovered the first step in the recycling of a crucial molecular tag which ensures the instructions encoded in our genes are correctly carried out. The study, published this week in the journal Cell, sheds new light on a proof-reading process that helps protect us from genetic diseases.

The translation of information from gene to protein in our cells is very important, but also error-prone. As errors can lead to diseases, several control mechanisms check for mistakes along the way. One such mechanism, called nonsense-mediated decay (NMD), is based on a molecular tag that is attached to messenger RNAs, an intermediate step in the translation from DNA to protein. The tag, called exon-junction complex (EJC), tells the NMD machinery if an RNA is faulty, potentially dangerous and should be degraded. Overall, a cell would need to mark around 400,000 sites with EJCs, but it only has 10,000 copies of one of the marker's components. This means EJCs must be broken down as soon as possible, so that their components can be re-used.

Researchers in the groups of Matthias Hentze, associate director of EMBL, and Andreas Kulozik at the University Clinic Heidelberg discovered that a protein called PYM is responsible for the disassembly and recycling of EJCs.

“Our results were very surprising,” says Niels Gehring, who carried out the research. “Everybody had assumed that ribosomes, the large structures that carry out protein assembly, simply iron out the EJCs as they pass. Now we see that this is not quite right, because without PYM EJC disassembly is impaired.”

Although PYM can be found on its own in the cell, it tends to associate with ribosomes. This explains why - and how - EJCs are removed when the ribosome goes by, and could also ensure that they are not removed too early. If that happened, NMD would be compromised, as the proofreading machinery would have no markers to guide it. This in turn could have wider consequences, as NMD influences how diseases such as thalassaemia, Duchenne's muscular dystrophy and cystic fibrosis manifest themselves.

“The new insights fill an important gap in the basic understanding of a vital cellular process,” says Hentze. “But they also have medical implications. Ultimately we would like to find ways to modulate NMD pharmacologically to influence the development and course of genetic diseases.”

The research was conducted in the joint Molecular Medicine Partnership Unit (MMPU), a collaboration between EMBL and Heidelberg University. “The MMPU bridges the gap between basic and clinical research. The constant cross-fertilisation between biologists and medical scientists guides our studies and often leads to discoveries that are applicable to medicine,” says Kulozik, medical director and professor of pediatrics at Heidelberg University.

Anna-Lynn Wegener | EMBL
Further information:
http://www.embl.org/aboutus/news/press/2009/01may09/index.html

More articles from Life Sciences:

nachricht Ion treatments for cardiac arrhythmia — Non-invasive alternative to catheter-based surgery
20.01.2017 | GSI Helmholtzzentrum für Schwerionenforschung GmbH

nachricht Seeking structure with metagenome sequences
20.01.2017 | DOE/Joint Genome Institute

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Traffic jam in empty space

New success for Konstanz physicists in studying the quantum vacuum

An important step towards a completely new experimental access to quantum physics has been made at University of Konstanz. The team of scientists headed by...

Im Focus: How gut bacteria can make us ill

HZI researchers decipher infection mechanisms of Yersinia and immune responses of the host

Yersiniae cause severe intestinal infections. Studies using Yersinia pseudotuberculosis as a model organism aim to elucidate the infection mechanisms of these...

Im Focus: Interfacial Superconductivity: Magnetic and superconducting order revealed simultaneously

Researchers from the University of Hamburg in Germany, in collaboration with colleagues from the University of Aarhus in Denmark, have synthesized a new superconducting material by growing a few layers of an antiferromagnetic transition-metal chalcogenide on a bismuth-based topological insulator, both being non-superconducting materials.

While superconductivity and magnetism are generally believed to be mutually exclusive, surprisingly, in this new material, superconducting correlations...

Im Focus: Studying fundamental particles in materials

Laser-driving of semimetals allows creating novel quasiparticle states within condensed matter systems and switching between different states on ultrafast time scales

Studying properties of fundamental particles in condensed matter systems is a promising approach to quantum field theory. Quasiparticles offer the opportunity...

Im Focus: Designing Architecture with Solar Building Envelopes

Among the general public, solar thermal energy is currently associated with dark blue, rectangular collectors on building roofs. Technologies are needed for aesthetically high quality architecture which offer the architect more room for manoeuvre when it comes to low- and plus-energy buildings. With the “ArKol” project, researchers at Fraunhofer ISE together with partners are currently developing two façade collectors for solar thermal energy generation, which permit a high degree of design flexibility: a strip collector for opaque façade sections and a solar thermal blind for transparent sections. The current state of the two developments will be presented at the BAU 2017 trade fair.

As part of the “ArKol – development of architecturally highly integrated façade collectors with heat pipes” project, Fraunhofer ISE together with its partners...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Sustainable Water use in Agriculture in Eastern Europe and Central Asia

19.01.2017 | Event News

12V, 48V, high-voltage – trends in E/E automotive architecture

10.01.2017 | Event News

2nd Conference on Non-Textual Information on 10 and 11 May 2017 in Hannover

09.01.2017 | Event News

 
Latest News

Helmholtz International Fellow Award for Sarah Amalia Teichmann

20.01.2017 | Awards Funding

An innovative high-performance material: biofibers made from green lacewing silk

20.01.2017 | Materials Sciences

Ion treatments for cardiac arrhythmia — Non-invasive alternative to catheter-based surgery

20.01.2017 | Life Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>