Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

First mouse model for ALS dementia

25.09.2014

Now scientists can directly view drugs' effect on brain

The first animal model for ALS dementia, a form of ALS that also damages the brain, has been developed by Northwestern Medicine® scientists. The advance will allow researchers to directly see the brains of living mice, under anesthesia, at the microscopic level. This will allow direct monitoring of test drugs to determine if they work.

This is one of the latest research findings since the ALS Ice Bucket Challenge heightened interest in the disease and the need for expanded research and funding.

"This new model will allow rapid testing and direct monitoring of drugs in real time," said Northwestern scientist and study senior author Teepu Siddique, M.D. "This will allow scientists to move quickly and accelerate the testing of drug therapies."

The new mouse model has the pathological hallmarks of the disease in humans with mutations in the genes for UBQLN2 (ubliqulin 2) and SQSTM1 (P62) that Siddique and colleagues identified in 2011. That pathology was linked to all forms of ALS and ALS/dementia.

Siddique and Han-Xiang Deng, M.D., the corresponding authors on the paper, said they have reproduced behavioral, neurophysiological and pathological changes in a mouse that mimic this form of dementia associated with ALS (amyotrophic lateral sclerosis).

Siddique is the Les Turner ALS Foundation/Herbert C. Wenske Professor of neurology at Northwestern University Feinberg School of Medicine and a neurologist at Northwestern Memorial Hospital. Deng is a research professor in neurology at Feinberg.

The study was published Sept. 22 in the Proceedings of the National Academy of Sciences.

It's been difficult for scientists to reproduce the genetic mutations of ALS, especially ALS/dementia in animal models, Siddique noted, which has hampered drug therapy testing.

Five percent or more of ALS cases, also known as Lou Gehrig's Disease, also have ALS/dementia.

"ALS with dementia is an even more vicious disease than ALS alone because it attacks the brain causing changes in behavior and language well as paralysis," Siddique said.

ALS affects an estimated 350,000 people worldwide, with an average survival of three years. In this progressive neurological disorder, the degeneration of neurons leads to muscle weakness and impaired speaking, swallowing and breathing, eventually causing paralysis and death. The associated dementia affects behavior and may affect decision-making, judgment, insight and language.

###

Other Northwestern authors on the paper include first authors George H. Gorrie, Faisal Fecto, Daniel Radzicki, Craig Weiss, Yong Shi, Hongxin Dong, Hong Zhai, Ronggen Fu, Erdong Liu, Sisi Li, Hasan Arrat, Eileen H. Bigio, John F. Disterhoft, Marco Martina and Enrico Mugnaini. Gorrie is now a consultant neurologist in Scotland.

The study was supported by the National Institute of Neurological Disorders and Stroke grants NS050641, NS078504, NS070142, NS081474, NS064091, NS009904, NS078287, and the National Institute on Aging grants AG20506 and AG13854, both of the National Institutes of Health, the Les Turner ALS Foundation, the Vena E. Schaff ALS Research Fund, a Harold Post Research Professorship, the Herbert and Florence C. Wenske Foundation and the Les Turner ALS Foundation/Herbert C. Wenske Foundation Professorship and others.

marla Paul | Eurek Alert!
Further information:
http://www.northwestern.edu/

More articles from Health and Medicine:

nachricht Satellites, airport visibility readings shed light on troops' exposure to air pollution
09.12.2016 | Veterans Affairs Research Communications

nachricht Oxygen can wake up dormant bacteria for antibiotic attacks
08.12.2016 | Penn State

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

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

Im Focus: Electron highway inside crystal

Physicists of the University of Würzburg have made an astonishing discovery in a specific type of topological insulators. The effect is due to the structure of the materials used. The researchers have now published their work in the journal Science.

Topological insulators are currently the hot topic in physics according to the newspaper Neue Zürcher Zeitung. Only a few weeks ago, their importance was...

Im Focus: Significantly more productivity in USP lasers

In recent years, lasers with ultrashort pulses (USP) down to the femtosecond range have become established on an industrial scale. They could advance some applications with the much-lauded “cold ablation” – if that meant they would then achieve more throughput. A new generation of process engineering that will address this issue in particular will be discussed at the “4th UKP Workshop – Ultrafast Laser Technology” in April 2017.

Even back in the 1990s, scientists were comparing materials processing with nanosecond, picosecond and femtosesecond pulses. The result was surprising:...

Im Focus: Shape matters when light meets atom

Mapping the interaction of a single atom with a single photon may inform design of quantum devices

Have you ever wondered how you see the world? Vision is about photons of light, which are packets of energy, interacting with the atoms or molecules in what...

Im Focus: Novel silicon etching technique crafts 3-D gradient refractive index micro-optics

A multi-institutional research collaboration has created a novel approach for fabricating three-dimensional micro-optics through the shape-defined formation of porous silicon (PSi), with broad impacts in integrated optoelectronics, imaging, and photovoltaics.

Working with colleagues at Stanford and The Dow Chemical Company, researchers at the University of Illinois at Urbana-Champaign fabricated 3-D birefringent...

Im Focus: Quantum Particles Form Droplets

In experiments with magnetic atoms conducted at extremely low temperatures, scientists have demonstrated a unique phase of matter: The atoms form a new type of quantum liquid or quantum droplet state. These so called quantum droplets may preserve their form in absence of external confinement because of quantum effects. The joint team of experimental physicists from Innsbruck and theoretical physicists from Hannover report on their findings in the journal Physical Review X.

“Our Quantum droplets are in the gas phase but they still drop like a rock,” explains experimental physicist Francesca Ferlaino when talking about the...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ICTM Conference 2017: Production technology for turbomachine manufacturing of the future

16.11.2016 | Event News

Innovation Day Laser Technology – Laser Additive Manufacturing

01.11.2016 | Event News

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

14.10.2016 | Event News

 
Latest News

Researchers identify potentially druggable mutant p53 proteins that promote cancer growth

09.12.2016 | Life Sciences

Scientists produce a new roadmap for guiding development & conservation in the Amazon

09.12.2016 | Ecology, The Environment and Conservation

Satellites, airport visibility readings shed light on troops' exposure to air pollution

09.12.2016 | Health and Medicine

VideoLinks
B2B-VideoLinks
More VideoLinks >>>