Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

A new cellular garbage control pathway with relevance for human neurodegenerative diseases

18.07.2014

Several human neurodegenerative diseases, including Alzheimer’s, Parkinson’s and Huntington’s disease but also ageing, are linked to an accumulation of abnormal and aggregated proteins in cells.

Cellular “garbage” can be removed from cells by sweeping them to a cellular recycling station known as the lysosome. Scientists at the Max Planck Institute of Biochemistry in Martinsried, Germany, now discovered a new family of helper proteins that recognize labeled cellular protein waste and guide them efficiently to the lysosome for destruction and subsequent recycling into their reusable compounds.


The newly identified proteins, termed CUET proteins (shown in red), recognize toxic protein aggregates and target the whole complex to the cellular waste disposal and recycling station, the lysosome.

Illustration: Stefan Jentsch / Copyright: MPI of Biochemistry

Proteins, the components of our body that execute, control and organize basically all functions in our cells, are made out of strings of amino acids, which – like an origami - are folded into specific and complex three-dimensional structures according to their desired functions.

However, since folding and maintaining of such structures is highly sensitive to cellular or environmental stress, proteins can potentially misfold or form clumps (aggregates). Such undesired protein waste can be toxic for cells and may even lead to cell death. Because several human neurodegenerative diseases are known to be linked to an accumulation of abnormal protein aggregates, basic science aimed to understand how cells remove cellular garbage is elementary for designing strategies for a potential prevention or cure of such disorders.

Scientists in the laboratory of Stefan Jentsch at the MPIB now successfully used baker’s yeast for screening for new cellular waste disposal pathways. Kefeng Lu, a postdoctoral researcher from China, discovered a new class of helper proteins (termed CUET proteins) present both in yeast and humans that recognize cellular garbage earmarked for disposal by an attached label in the form of the ubiquitously existing protein known as “ubiquitin”.

Importantly, these newly identified helper proteins channel the cellular garbage by a “self-eating” pathway (autophagy) to the lysosome, a compartment of cells dedicated for destruction and recycling. The Max Planck scientists could also show that a toxic protein related to the abnormal, aggregate-forming protein “huntingtin” of patients with the neurodegenerative Huntington’s disease is efficiently destroyed by the newly identified pathway. Remarkably, this pathway seems specific for aggregated proteins like huntingtin and appears to be more potent than previously discovered cellular garbage disposal mechanisms.

Because the identified cellular disposal mechanism operates in yeast as well, the researches will now take full advantage of its powerful experimental possibilities to investigate this pathway further.

A detailed analysis of this mechanism will be crucial to understand how aggregate-forming proteins lead to human diseases and may help to develop concepts for possible disease preventions.

Original Publication:
K. Lu, I. Psakhye and S. Jentsch: Autophagic clearance of polyQ proteins mediated by the conserved CUET protein family. Cell, July 17, 2014.
DOI: 10.1016/j.cell.2014.05.048

Contact:
Prof. Dr. Stefan Jentsch
Molecular Cell Biology
Max Planck Institute of Biochemistry
Am Klopferspitz 18
82152 Martinsried
Germany
E-Mail: jentsch@biochem.mpg.de
http://www.biochem.mpg.de/jentsch

Anja Konschak
Public Relations
Max Planck Institute of Biochemistry
Am Klopferspitz 18
82152 Martinsried
Germany
Tel. +49 89 8578-2824
E-Mail: konschak@biochem.mpg.de
http://www.biochem.mpg.de/news

Weitere Informationen:

http://www.biochem.mpg.de/jentsch - Website of the Research Departement "Molecular Cell Biology" (Stefan Jentsch)
http://www.biochem.mpg.de/news/ueber_das_institut/forschungsbereiche/zellbiologi... - More press releases about the research of Stefan Jentsch

Anja Konschak | idw - Informationsdienst Wissenschaft

More articles from Life Sciences:

nachricht Wbp2 is a novel deafness gene
09.02.2016 | EMBO - excellence in life sciences

nachricht Laser instead of Reading Glasses?
09.02.2016 | Laser Zentrum Hannover e.V.

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Wbp2 is a novel deafness gene

Researchers at King’s College London and the Wellcome Trust Sanger Institute in the United Kingdom have for the first time demonstrated a direct link between the Wbp2 gene and progressive hearing loss. The scientists report that the loss of Wbp2 expression leads to progressive high-frequency hearing loss in mouse as well as in two clinical cases of children with deafness with no other obvious features. The results are published in EMBO Molecular Medicine.

The scientists have shown that hearing impairment is linked to hormonal signalling rather than to hair cell degeneration. Wbp2 is known as a transcriptional...

Im Focus: From allergens to anodes: Pollen derived battery electrodes

Pollens, the bane of allergy sufferers, could represent a boon for battery makers: Recent research has suggested their potential use as anodes in lithium-ion batteries.

"Our findings have demonstrated that renewable pollens could produce carbon architectures for anode applications in energy storage devices," said Vilas Pol, an...

Im Focus: Automated driving: Steering without limits

OmniSteer project to increase automobiles’ urban maneuverability begins with a € 3.4 million budget

Automobiles increase the mobility of their users. However, their maneuverability is pushed to the limit by cramped inner city conditions. Those who need to...

Im Focus: Microscopy: Nine at one blow

Advance in biomedical imaging: The University of Würzburg's Biocenter has enhanced fluorescence microscopy to label and visualise up to nine different cell structures simultaneously.

Fluorescence microscopy allows researchers to visualise biomolecules in cells. They label the molecules using fluorescent probes, excite them with light and...

Im Focus: NASA's ICESat-2 equipped with unique 3-D manufactured part

NASA's follow-on to the successful ICESat mission will employ a never-before-flown technique for determining the topography of ice sheets and the thickness of sea ice, but that won't be the only first for this mission.

Slated for launch in 2018, NASA's Ice, Cloud and land Elevation Satellite-2 (ICESat-2) also will carry a 3-D printed part made of polyetherketoneketone (PEKK),...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

AKL’16: Experience Laser Technology Live in Europe´s Largest Laser Application Center!

02.02.2016 | Event News

From intelligent knee braces to anti-theft backpacks

26.01.2016 | Event News

DATE 2016 Highlighting Automotive and Secure Systems

26.01.2016 | Event News

 
Latest News

Laser instead of Reading Glasses?

09.02.2016 | Life Sciences

Wbp2 is a novel deafness gene

09.02.2016 | Life Sciences

Ocean acidification makes coralline algae less robust

08.02.2016 | Earth Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>