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 Embryonic development: How do limbs develop from cells?
18.05.2018 | Humboldt-Universität zu Berlin

nachricht Reading histone modifications, an oncoprotein is modified in return
18.05.2018 | American Society for Biochemistry and Molecular Biology

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Explanation for puzzling quantum oscillations has been found

So-called quantum many-body scars allow quantum systems to stay out of equilibrium much longer, explaining experiment | Study published in Nature Physics

Recently, researchers from Harvard and MIT succeeded in trapping a record 53 atoms and individually controlling their quantum state, realizing what is called a...

Im Focus: Dozens of binaries from Milky Way's globular clusters could be detectable by LISA

Next-generation gravitational wave detector in space will complement LIGO on Earth

The historic first detection of gravitational waves from colliding black holes far outside our galaxy opened a new window to understanding the universe. A...

Im Focus: Entangled atoms shine in unison

A team led by Austrian experimental physicist Rainer Blatt has succeeded in characterizing the quantum entanglement of two spatially separated atoms by observing their light emission. This fundamental demonstration could lead to the development of highly sensitive optical gradiometers for the precise measurement of the gravitational field or the earth's magnetic field.

The age of quantum technology has long been heralded. Decades of research into the quantum world have led to the development of methods that make it possible...

Im Focus: Computer-Designed Customized Regenerative Heart Valves

Cardiovascular tissue engineering aims to treat heart disease with prostheses that grow and regenerate. Now, researchers from the University of Zurich, the Technical University Eindhoven and the Charité Berlin have successfully implanted regenerative heart valves, designed with the aid of computer simulations, into sheep for the first time.

Producing living tissue or organs based on human cells is one of the main research fields in regenerative medicine. Tissue engineering, which involves growing...

Im Focus: Light-induced superconductivity under high pressure

A team of scientists of the Max Planck Institute for the Structure and Dynamics of Matter (MPSD) at the Center for Free-Electron Laser Science in Hamburg investigated optically-induced superconductivity in the alkali-doped fulleride K3C60under high external pressures. This study allowed, on one hand, to uniquely assess the nature of the transient state as a superconducting phase. In addition, it unveiled the possibility to induce superconductivity in K3C60 at temperatures far above the -170 degrees Celsius hypothesized previously, and rather all the way to room temperature. The paper by Cantaluppi et al has been published in Nature Physics.

Unlike ordinary metals, superconductors have the unique capability of transporting electrical currents without any loss. Nowadays, their technological...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

Save the date: Forum European Neuroscience – 07-11 July 2018 in Berlin, Germany

02.05.2018 | Event News

Invitation to the upcoming "Current Topics in Bioinformatics: Big Data in Genomics and Medicine"

13.04.2018 | Event News

Unique scope of UV LED technologies and applications presented in Berlin: ICULTA-2018

12.04.2018 | Event News

 
Latest News

Supersonic waves may help electronics beat the heat

18.05.2018 | Power and Electrical Engineering

Keeping a Close Eye on Ice Loss

18.05.2018 | Information Technology

CrowdWater: An App for Flood Research

18.05.2018 | Information Technology

VideoLinks
Science & Research
Overview of more VideoLinks >>>