Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Molecular mechanism sheds light on neurodegenerative diseases

22.10.2004


Alzheimer’s. Parkinson’s. Lou Gehrig’s. Huntington’s. These neurodegenerative diseases exhibit loss of nerve function in different ways, from memory lapses to uncontrollable muscular movements, but it is now believed that these diseases share many common molecular mechanisms.



A team of Northwestern University scientists, led by Richard I. Morimoto, John Evans Professor of Biology, has made a key discovery toward understanding one of these mechanisms. In studying toxic proteins involved in Huntington’s disease, they discovered that the disease-causing protein severely interferes with the working of the proteasome, the cellular machine responsible for eliminating damaged proteins within the cell.

The findings, which could lead to an understanding of how to prevent neurodegenerative diseases and to the development of effective drugs, will be published Oct. 27 in The EMBO Journal, a publication of the European Molecular Biology Organization.


The proteasome is responsible for cell homeostasis. In healthy cells, proteins perform their function and then, with the help of the proteasome, disappear. If idle and damaged proteins remain, their presence can affect cell behavior.

Misfolded and damaged proteins are common to all human neurodegenerative diseases. They clump together to form toxic aggregates that destroy cell function and cause disease. Morimoto’s team is the first to demonstrate in living human cells and in real time that the toxic protein aggregates, in this case caused by mutant Huntingtin, bind to the proteasome machine irreversibly and prevent the complete degradation of the proteins. This evidence could help explain the disease process.

"We believe this suggests why Huntington’s disease is so destructive," said Morimoto. "Once bound, the toxic proteins do not release the proteasome. This interference with the normal clearance of proteins has a cumulative and amplifying negative effect. The proteins that are normally degraded build up."

The researchers’ data also show that the toxic proteins and proteasome are bound together in a close and stable fashion, indicating that the proteins are trapped within the proteasome. This could explain the negative consequences on the health of the cell in which disease builds over decades before symptoms result.

In addition to Morimoto, other authors on the EMBO paper are Carina I. Holmberg, a post-doctoral fellow and the paper’s lead author; Kwame N. Mensah, a graduate student; and Andreas Matouschek, associate professor of biochemistry, molecular biology and cell biology, from Northwestern University; and Kristine E. Staniszweski, a former graduate student at Northwestern.

Megan Fellman | EurekAlert!
Further information:
http://www.northwestern.edu

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Can we see monkeys from space? Emerging technologies to map biodiversity

An international team of scientists has proposed a new multi-disciplinary approach in which an array of new technologies will allow us to map biodiversity and the risks that wildlife is facing at the scale of whole landscapes. The findings are published in Nature Ecology and Evolution. This international research is led by the Kunming Institute of Zoology from China, University of East Anglia, University of Leicester and the Leibniz Institute for Zoo and Wildlife Research.

Using a combination of satellite and ground data, the team proposes that it is now possible to map biodiversity with an accuracy that has not been previously...

Im Focus: Climate satellite: Tracking methane with robust laser technology

Heatwaves in the Arctic, longer periods of vegetation in Europe, severe floods in West Africa – starting in 2021, scientists want to explore the emissions of the greenhouse gas methane with the German-French satellite MERLIN. This is made possible by a new robust laser system of the Fraunhofer Institute for Laser Technology ILT in Aachen, which achieves unprecedented measurement accuracy.

Methane is primarily the result of the decomposition of organic matter. The gas has a 25 times greater warming potential than carbon dioxide, but is not as...

Im Focus: How protons move through a fuel cell

Hydrogen is regarded as the energy source of the future: It is produced with solar power and can be used to generate heat and electricity in fuel cells. Empa researchers have now succeeded in decoding the movement of hydrogen ions in crystals – a key step towards more efficient energy conversion in the hydrogen industry of tomorrow.

As charge carriers, electrons and ions play the leading role in electrochemical energy storage devices and converters such as batteries and fuel cells. Proton...

Im Focus: A unique data centre for cosmological simulations

Scientists from the Excellence Cluster Universe at the Ludwig-Maximilians-Universität Munich have establised "Cosmowebportal", a unique data centre for cosmological simulations located at the Leibniz Supercomputing Centre (LRZ) of the Bavarian Academy of Sciences. The complete results of a series of large hydrodynamical cosmological simulations are available, with data volumes typically exceeding several hundred terabytes. Scientists worldwide can interactively explore these complex simulations via a web interface and directly access the results.

With current telescopes, scientists can observe our Universe’s galaxies and galaxy clusters and their distribution along an invisible cosmic web. From the...

Im Focus: Scientists develop molecular thermometer for contactless measurement using infrared light

Temperature measurements possible even on the smallest scale / Molecular ruby for use in material sciences, biology, and medicine

Chemists at Johannes Gutenberg University Mainz (JGU) in cooperation with researchers of the German Federal Institute for Materials Research and Testing (BAM)...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Plants are networkers

19.06.2017 | Event News

Digital Survival Training for Executives

13.06.2017 | Event News

Global Learning Council Summit 2017

13.06.2017 | Event News

 
Latest News

Supersensitive through quantum entanglement

28.06.2017 | Physics and Astronomy

X-ray photoelectron spectroscopy under real ambient pressure conditions

28.06.2017 | Physics and Astronomy

Mice provide insight into genetics of autism spectrum disorders

28.06.2017 | Health and Medicine

VideoLinks
B2B-VideoLinks
More VideoLinks >>>