Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

LSUHSC research discovery may block ALS disease process

20.04.2011
In the first animal model of Amyotrophic Lateral Sclerosis (ALS), developed by Dr. Udai Pandey, Assistant Professor of Genetics at LSU Health Sciences Center New Orleans, Dr. Pandey's lab has found in fruit flies that blocking the abnormal movement of a protein made by a mutated gene called FUS also blocks the disease process. The research is available online in the Advanced Access section of the journal Human Molecular Genetics website, posted on April 12, 2011. It will be published in an upcoming issue of the journal.

The fruit flies were engineered to carry and express a mutated human FUS gene. This mutated FUS gene has been shown to be one of the major causes of both familial and sporadic ALS. In the fruit flies, the resulting neurodegeneration impairs their ability to walk or climb and the defect is also easily visualized in the structure of their eyes. In addition, the flies carrying the defective FUS gene demonstrate hallmarks of the human disease, such as an age-dependent degeneration of neurons, accumulation of abnormal proteins and a decrease in life span.

Also as seen in human ALS patients, the disease-causing FUS protein that's formed from the gene abnormally moves to the cytoplasm rather than staying in the cell nucleus. Dr. Pandey's lab found that blocking this abnormal migration could block the disease process. All these features make the fly model a valuable resource for performing drug screens to identify drugs that could modify the effects of the mutated gene.

"These findings prompt us to look for drugs that can help in keeping the defective FUS protein in the nucleus as a potential therapeutic intervention" notes Pandey. "The fly model is an inexpensive and fast way to study many human diseases such as cancer, Alzheimer's disease and Parkinson's disease. Many basic biological processes are well conserved between humans and fruit flies, and nearly 75% of human disease-causing genes are believed to have a functional partner (homolog) in the fly that makes these small animals a highly tractable model system."

Dr. Pandey's group found that normal FUS interacts with another major human ALS-linked protein TDP-43, but mutated FUS interacts abnormally with normal TDP-43. Mutations in the TDP-43 gene have also been found to cause ALS. Interestingly, these two ALS-linked proteins do not seem to interact if trapped in the nucleus.

According to the National Institutes of Health, Amyotrophic Lateral Sclerosis, sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons. Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from them to particular muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twitch (fasciculations). Eventually, the ability of the brain to start and control voluntary movement is lost.

"Our next goal is to identify other factors such as proteins or RNA that mutant forms of FUS target so that we can get more insights into the disease mechanisms" said Nicholas Lanson Jr., an LSUHSC research associate and first author of the paper.

The Robert Packard Center for ALS at Johns Hopkins generously funded Dr. Pandey's lab in developing a fruit fly model of ALS. Nicholas Lanson Jr., Astha Maltare (LSUHSC), Rebecca Smith, J Paul Taylor (St. Jude Children's Hospital), Ji Ham Kim and Thomas E Lloyd (Johns Hopkins Medical Center) are other contributors on this publication.

LSU Health Sciences Center New Orleans educates Louisiana's health care professionals. The state's academic health leader, LSUHSC New Orleans consists of a School of Medicine, the state's only School of Dentistry, Louisiana's only public School of Public Health, Schools of Allied Health Professions and Graduate Studies, and the only School of Nursing within an academic health center in the State of Louisiana. LSUHSC faculty take care of patients in public and private settings in the region, conduct research that improves the quality of life and generates jobs and economic impact, and perform service and outreach activities spanning the State. To learn more, visit http://www.lsuhsc.edu and http://www.twitter.com/LSUHSCHealth.

Leslie Capo | EurekAlert!
Further information:
http://www.lsuhsc.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 >>>