Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Landmark study identifies large number of new proteins implicated in Huntington's disease

14.05.2007
Buck Institute faculty leads large scale screening of protein interactions to identify drug targets for possible treatment of incurable disease

Researchers from four organizations have identified more than 200 new proteins that bind to normal and mutant forms of the protein that causes Huntington’s disease (HD). HD is a fatal inherited disease that affects 30,000 Americans annually by laying waste to their nervous system. The research was led by Buck Institute faculty member Robert E. Hughes, PhD. Results of the study, which may facilitate the discovery of an effective treatment for HD, will be published in the May 11 edition of PLoS Genetics, an online, open-source journal, enabling scientists from around the world to take advantage of the findings immediately.

The work, which involved high-tech screening of the human genome and proteome, was unprecedented both in terms of its scale and in the way the protein interactions were validated in a genetic model of the disease. By conducting additional experiments in fruit flies genetically altered to express features of human HD, scientists showed that changing the expression of these interacting proteins affected the degree of damage seen in the fly neurons. This indicates that a significant number of the proteins might be potential drug targets for HD.

Now that researchers have discovered the interacting proteins using human libraries and human protein extracts and tested them in the fly, Hughes says the next step is to bring the research back into the mammalian world. The new genes and proteins discovered in this study are being screened and analyzed in cultured mammalian cells; the ones that show activity in ongoing experiments will be tested in mouse models of HD.

"Here at the Buck Institute, we’re going to be focusing on a few dozen proteins," said Hughes. "Effective follow-up on any target protein depends, in large part, on how much expertise a scientist has with that target. We are hoping that researchers will look at this study and that those with specific expertise in a particular protein will move forward with their own inquiries."

The work was supported by HD advocacy organizations. "We are very excited about this significant discovery," said Allan Tobin, PhD, Senior Scientific Advisor to the High Q Foundation and CHDI, Inc. "This work helps define and refine possible therapeutic targets for a disease that lacks thorough understanding." Tobin added, "We are pleased this study is being published in an open-access journal, which makes it easier for scientists at other organizations to get to work on following up on this landmark discovery." Traditional peer-reviewed journals usually require scientists to pay a fee to access study results.

Tobin added that the need for further scientific inquiry is urgent. There is currently no effective treatment or cure for HD, which is typically characterized by involuntary movements and dementia. The disease slowly diminishes a person’s ability to move, think and communicate. Those affected eventually become totally dependent on others for their care and usually die from complications such as choking, heart failure or infection. The disease is hereditary; each child of a person with HD has a 50/50 chance of inheriting the fatal gene. Approximately 200,000 Americans are believed to be at risk of developing HD, a disease that affects as many people as hemophilia, cystic fibrosis or muscular dystrophy. The symptoms of HD typically begin to appear in mid-life, although the progression of the disease varies among individuals and within the same family.

Kris Rebillot | EurekAlert!
Further information:
http://www.buckinstitute.org

More articles from Studies and Analyses:

nachricht Amputees can learn to control a robotic arm with their minds
28.11.2017 | University of Chicago Medical Center

nachricht The importance of biodiversity in forests could increase due to climate change
17.11.2017 | Deutsches Zentrum für integrative Biodiversitätsforschung (iDiv) Halle-Jena-Leipzig

All articles from Studies and Analyses >>>

The most recent press releases about innovation >>>

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

Im Focus: Long-lived storage of a photonic qubit for worldwide teleportation

MPQ scientists achieve long storage times for photonic quantum bits which break the lower bound for direct teleportation in a global quantum network.

Concerning the development of quantum memories for the realization of global quantum networks, scientists of the Quantum Dynamics Division led by Professor...

Im Focus: Electromagnetic water cloak eliminates drag and wake

Detailed calculations show water cloaks are feasible with today's technology

Researchers have developed a water cloaking concept based on electromagnetic forces that could eliminate an object's wake, greatly reducing its drag while...

Im Focus: Scientists channel graphene to understand filtration and ion transport into cells

Tiny pores at a cell's entryway act as miniature bouncers, letting in some electrically charged atoms--ions--but blocking others. Operating as exquisitely sensitive filters, these "ion channels" play a critical role in biological functions such as muscle contraction and the firing of brain cells.

To rapidly transport the right ions through the cell membrane, the tiny channels rely on a complex interplay between the ions and surrounding molecules,...

Im Focus: Towards data storage at the single molecule level

The miniaturization of the current technology of storage media is hindered by fundamental limits of quantum mechanics. A new approach consists in using so-called spin-crossover molecules as the smallest possible storage unit. Similar to normal hard drives, these special molecules can save information via their magnetic state. A research team from Kiel University has now managed to successfully place a new class of spin-crossover molecules onto a surface and to improve the molecule’s storage capacity. The storage density of conventional hard drives could therefore theoretically be increased by more than one hundred fold. The study has been published in the scientific journal Nano Letters.

Over the past few years, the building blocks of storage media have gotten ever smaller. But further miniaturization of the current technology is hindered by...

Im Focus: Successful Mechanical Testing of Nanowires

With innovative experiments, researchers at the Helmholtz-Zentrums Geesthacht and the Technical University Hamburg unravel why tiny metallic structures are extremely strong

Light-weight and simultaneously strong – porous metallic nanomaterials promise interesting applications as, for instance, for future aeroplanes with enhanced...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

See, understand and experience the work of the future

11.12.2017 | Event News

Innovative strategies to tackle parasitic worms

08.12.2017 | Event News

AKL’18: The opportunities and challenges of digitalization in the laser industry

07.12.2017 | Event News

 
Latest News

A whole-body approach to understanding chemosensory cells

13.12.2017 | Health and Medicine

Water without windows: Capturing water vapor inside an electron microscope

13.12.2017 | Physics and Astronomy

Cellular Self-Digestion Process Triggers Autoimmune Disease

13.12.2017 | Life Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>