Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:


Molecular mechanism sheds light on neurodegenerative diseases


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:

More articles from Life Sciences:

nachricht Strong, steady forces at work during cell division
20.10.2016 | University of Massachusetts at Amherst

nachricht Disturbance wanted
20.10.2016 | Max Delbrück Center for Molecular Medicine in the Helmholtz Association

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: New 3-D wiring technique brings scalable quantum computers closer to reality

Researchers from the Institute for Quantum Computing (IQC) at the University of Waterloo led the development of a new extensible wiring technique capable of controlling superconducting quantum bits, representing a significant step towards to the realization of a scalable quantum computer.

"The quantum socket is a wiring method that uses three-dimensional wires based on spring-loaded pins to address individual qubits," said Jeremy Béjanin, a PhD...

Im Focus: Scientists develop a semiconductor nanocomposite material that moves in response to light

In a paper in Scientific Reports, a research team at Worcester Polytechnic Institute describes a novel light-activated phenomenon that could become the basis for applications as diverse as microscopic robotic grippers and more efficient solar cells.

A research team at Worcester Polytechnic Institute (WPI) has developed a revolutionary, light-activated semiconductor nanocomposite material that can be used...

Im Focus: Diamonds aren't forever: Sandia, Harvard team create first quantum computer bridge

By forcefully embedding two silicon atoms in a diamond matrix, Sandia researchers have demonstrated for the first time on a single chip all the components needed to create a quantum bridge to link quantum computers together.

"People have already built small quantum computers," says Sandia researcher Ryan Camacho. "Maybe the first useful one won't be a single giant quantum computer...

Im Focus: New Products - Highlights of COMPAMED 2016

COMPAMED has become the leading international marketplace for suppliers of medical manufacturing. The trade fair, which takes place every November and is co-located to MEDICA in Dusseldorf, has been steadily growing over the past years and shows that medical technology remains a rapidly growing market.

In 2016, the joint pavilion by the IVAM Microtechnology Network, the Product Market “High-tech for Medical Devices”, will be located in Hall 8a again and will...

Im Focus: Ultra-thin ferroelectric material for next-generation electronics

'Ferroelectric' materials can switch between different states of electrical polarization in response to an external electric field. This flexibility means they show promise for many applications, for example in electronic devices and computer memory. Current ferroelectric materials are highly valued for their thermal and chemical stability and rapid electro-mechanical responses, but creating a material that is scalable down to the tiny sizes needed for technologies like silicon-based semiconductors (Si-based CMOS) has proven challenging.

Now, Hiroshi Funakubo and co-workers at the Tokyo Institute of Technology, in collaboration with researchers across Japan, have conducted experiments to...

All Focus news of the innovation-report >>>



Event News

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

14.10.2016 | Event News

Agricultural Trade Developments and Potentials in Central Asia and the South Caucasus

14.10.2016 | Event News

World Health Summit – Day Three: A Call to Action

12.10.2016 | Event News

Latest News

Innovative technique for shaping light could solve bandwidth crunch

20.10.2016 | Physics and Astronomy

Finding the lightest superdeformed triaxial atomic nucleus

20.10.2016 | Physics and Astronomy

NASA's MAVEN mission observes ups and downs of water escape from Mars

20.10.2016 | Physics and Astronomy

More VideoLinks >>>