Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Einstein scientists discover cause and possible treatments for hereditary movement disorder

03.03.2006


Researchers at the Albert Einstein College of Medicine of Yeshiva University have discovered the underlying cause of a type of ataxia, hereditary disorders characterized by poor balance, loss of posture and difficulty performing rapid coordinated movement. Their work also led to a drug that significantly improved the motor coordination in mice with ataxia--a finding that could lead to better therapies for the disease. The study appears in the March issue of Nature Neuroscience and was featured in the publication’s advance online edition.



The research, led by Dr. Kamran Khodakhah, associate professor in the department of neuroscience at Einstein, focused on a type of ataxia called episodic ataxia type-2. It results from gene mutations that affect calcium channels, which are involved in releasing neurotransmitters in the brain and regulating excitability in neurons. Episodic ataxia type-2 was thought to be due to impaired transmission of neurotransmitters, but the Einstein scientists suspected that something else was going on.

They studied specialized cells in the brain’s cerebellum called Purkinje cells, which are rich in calcium channels. Purkinje cells help coordinate movement by acting as information clearinghouses: They take in sensory and other inputs relayed to them by more than 150,000 excitatory and inhibitory synaptic inputs, combine them with the cello’s own intrinsic activity or "pacemaking," and then send out the signals necessary for motor coordination.


The researchers investigated whether ataxia might be due to a reduction in the precision of the intrinsic pacemaking by Purkinje cells. Studying a number of mouse models of ataxia type-2, they found a gene-dependent loss of the precision of pacemaking in Purkinje cells, which prevented them from accurately accounting for the strength and timing of synaptic inputs when sending out signals directing muscle movement.

This loss of pacemaking precision was traced to reduced activity of calcium-activated potassium channels in Purkinje cells -- a direct consequence of the reduced activity of calcium channels in these disorders. Einstein researchers were able to remedy this problem with a drug called 1-ethyl-2-benzimidazolinone (EBIO). When EBIO was infused into the brains of ataxic mice, the mice’s motor coordination improved significantly.

"These calcium-activated potassium channels proved to be a potent therapeutic target, since chronically activating them with EBIO definitely improved the motor performance of these ataxic mice," says Dr. Khodakhah, who was senior author of the study. "We don’t really have effective treatments for these types of ataxia, so we’re hopeful that our findings will lead to drugs that will improve the lives of people with this condition."

Dr Khodakhah has established collaborations with two neurologists, Dr Joanna Jen (UCLA) and Dr Michael Strupp (Germany) to explore the potential use of similar drugs in patients. The other Einstein researchers involved in the study were Joy T. Walter, Karina Alvina, Mary D. Womack and Carolyn Chevez.

Karen Gardner | EurekAlert!
Further information:
http://www.aecom.yu.edu

More articles from Life Sciences:

nachricht New risk factors for anxiety disorders
24.02.2017 | Julius-Maximilians-Universität Würzburg

nachricht Stingless bees have their nests protected by soldiers
24.02.2017 | Johannes Gutenberg-Universität Mainz

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Breakthrough with a chain of gold atoms

In the field of nanoscience, an international team of physicists with participants from Konstanz has achieved a breakthrough in understanding heat transport

In the field of nanoscience, an international team of physicists with participants from Konstanz has achieved a breakthrough in understanding heat transport

Im Focus: DNA repair: a new letter in the cell alphabet

Results reveal how discoveries may be hidden in scientific “blind spots”

Cells need to repair damaged DNA in our genes to prevent the development of cancer and other diseases. Our cells therefore activate and send “repair-proteins”...

Im Focus: Dresdner scientists print tomorrow’s world

The Fraunhofer IWS Dresden and Technische Universität Dresden inaugurated their jointly operated Center for Additive Manufacturing Dresden (AMCD) with a festive ceremony on February 7, 2017. Scientists from various disciplines perform research on materials, additive manufacturing processes and innovative technologies, which build up components in a layer by layer process. This technology opens up new horizons for component design and combinations of functions. For example during fabrication, electrical conductors and sensors are already able to be additively manufactured into components. They provide information about stress conditions of a product during operation.

The 3D-printing technology, or additive manufacturing as it is often called, has long made the step out of scientific research laboratories into industrial...

Im Focus: Mimicking nature's cellular architectures via 3-D printing

Research offers new level of control over the structure of 3-D printed materials

Nature does amazing things with limited design materials. Grass, for example, can support its own weight, resist strong wind loads, and recover after being...

Im Focus: Three Magnetic States for Each Hole

Nanometer-scale magnetic perforated grids could create new possibilities for computing. Together with international colleagues, scientists from the Helmholtz Zentrum Dresden-Rossendorf (HZDR) have shown how a cobalt grid can be reliably programmed at room temperature. In addition they discovered that for every hole ("antidot") three magnetic states can be configured. The results have been published in the journal "Scientific Reports".

Physicist Dr. Rantej Bali from the HZDR, together with scientists from Singapore and Australia, designed a special grid structure in a thin layer of cobalt in...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Booth and panel discussion – The Lindau Nobel Laureate Meetings at the AAAS 2017 Annual Meeting

13.02.2017 | Event News

Complex Loading versus Hidden Reserves

10.02.2017 | Event News

International Conference on Crystal Growth in Freiburg

09.02.2017 | Event News

 
Latest News

Stingless bees have their nests protected by soldiers

24.02.2017 | Life Sciences

New risk factors for anxiety disorders

24.02.2017 | Life Sciences

MWC 2017: 5G Capital Berlin

24.02.2017 | Trade Fair News

VideoLinks
B2B-VideoLinks
More VideoLinks >>>