Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

New insights into how Huntington’s disease attacks the brain

06.05.2005


UCLA Neuropsychiatric Institute study reveals new approach



Scientific theory holds that Huntington’s disease (HD) is caused by a mutant protein that arises within brain cells and kills them, triggering the genetic neurological disorder. Now a new UCLA Neuropsychiatric Institute study reveals the first strong evidence that the mutant protein also elicits toxic interactions from neighboring cells to provoke the fatal brain disorder. The May 5 edition of Neuron reports the findings.

"This is really important because most current disease models and drug development efforts rely on the assumption that Huntington’s disease arises from within the target brain cells," explained Dr. William Yang, assistant professor at the UCLA Neuropsychiatric Institute and a member of the Brain Research Institute.


"Our model is the first to show that mutant HD proteins exert their influence on brain cells located near the target cells," he said. "These neighboring cells then interact with the target cells to spark disease."

To pinpoint the disorder’s cellular origin, UCLA researchers developed two sets of mice with the human HD gene mutation. The first group was engineered to trigger production of the mutant HD protein throughout the brain. The second set of mice produced the mutant HD protein only in the target brain cells.

The scientists reasoned that if the mutant protein triggered the disease only from within the target cells, the second set of mice would display significant signs of the disorder. If HD required toxic interactions among cells throughout the brain, however, these same mice would show little or no signs of the disorder.

When comparing the two groups, the UCLA team discovered that the first set of mice demonstrated problems with motor control and showed visible degeneration of the target brain cells. In contrast, the second set of mice showed little signs of the disease.

"This is the first direct genetic evidence to demonstrate that abnormal interactions between cells can significantly contribute to brain cell death in a living mouse model of Huntington’s disease," said Yang.

Yang’s team is now trying to pinpoint which of the neighboring cells generate Huntington’s disease.

"Our next step will be determining how neighboring cells influence target cells and cause their death," he said. "Once we understand how these cells interact, the knowledge may lead to new therapeutic strategies to treat Huntington’s disease."

Huntington’s disease is a genetic brain disorder that usually strikes in mid-life, but can also attack the elderly and children as young as 2. Slowly depriving a person of their ability to think, speak, walk and swallow, the disease robs the person of their independence, leading to death within 10 to 25 years.

Every carrier of the HD gene mutation will develop the disease. Each child of a parent with Huntington’s disease possesses a 50 percent risk of inheriting the illness. In the United States, the disease strikes 30,000 people and places another 150,000 persons at risk. The disorder affects males and females equally and crosses all ethnic and racial boundaries.

Elaine Schmidt | EurekAlert!
Further information:
http://www.mednet.ucla.edu

More articles from Life Sciences:

nachricht One step closer to reality
20.04.2018 | Max-Planck-Institut für Entwicklungsbiologie

nachricht The dark side of cichlid fish: from cannibal to caregiver
20.04.2018 | Veterinärmedizinische Universität Wien

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Spider silk key to new bone-fixing composite

University of Connecticut researchers have created a biodegradable composite made of silk fibers that can be used to repair broken load-bearing bones without the complications sometimes presented by other materials.

Repairing major load-bearing bones such as those in the leg can be a long and uncomfortable process.

Im Focus: Writing and deleting magnets with lasers

Study published in the journal ACS Applied Materials & Interfaces is the outcome of an international effort that included teams from Dresden and Berlin in Germany, and the US.

Scientists at the Helmholtz-Zentrum Dresden-Rossendorf (HZDR) together with colleagues from the Helmholtz-Zentrum Berlin (HZB) and the University of Virginia...

Im Focus: Gamma-ray flashes from plasma filaments

Novel highly efficient and brilliant gamma-ray source: Based on model calculations, physicists of the Max PIanck Institute for Nuclear Physics in Heidelberg propose a novel method for an efficient high-brilliance gamma-ray source. A giant collimated gamma-ray pulse is generated from the interaction of a dense ultra-relativistic electron beam with a thin solid conductor. Energetic gamma-rays are copiously produced as the electron beam splits into filaments while propagating across the conductor. The resulting gamma-ray energy and flux enable novel experiments in nuclear and fundamental physics.

The typical wavelength of light interacting with an object of the microcosm scales with the size of this object. For atoms, this ranges from visible light to...

Im Focus: Basel researchers succeed in cultivating cartilage from stem cells

Stable joint cartilage can be produced from adult stem cells originating from bone marrow. This is made possible by inducing specific molecular processes occurring during embryonic cartilage formation, as researchers from the University and University Hospital of Basel report in the scientific journal PNAS.

Certain mesenchymal stem/stromal cells from the bone marrow of adults are considered extremely promising for skeletal tissue regeneration. These adult stem...

Im Focus: Like a wedge in a hinge

Researchers lay groundwork to tailor drugs for new targets in cancer therapy

In the fight against cancer, scientists are developing new drugs to hit tumor cells at so far unused weak points. Such a “sore spot” is the protein complex...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
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

IWOLIA: A conference bringing together German Industrie 4.0 and French Industrie du Futur

09.04.2018 | Event News

 
Latest News

Magnetic nano-imaging on a table top

20.04.2018 | Physics and Astronomy

Start of work for the world's largest electric truck

20.04.2018 | Interdisciplinary Research

Atoms may hum a tune from grand cosmic symphony

20.04.2018 | Physics and Astronomy

VideoLinks
Science & Research
Overview of more VideoLinks >>>