Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Mouse model of neuromuscular disease identifies key player in motor neuron death

04.03.2004


Scientists have created a new mouse model for spinal and bulbar muscular atrophy (SBMA), a disease characterized by adult-onset progressive weakness and degeneration of limb muscles, often resulting in the patient being confined to a wheel chair. SBMA causes the death of cells called motor neurons that control muscle function. The study, published in the March 4 issue of Neuron, presents a clearer picture of the pathology underlying SBMA and associated diseases and even points to a possible therapeutic strategy for this debilitating condition and for more common motor neuron diseases, such as amyotrophic lateral sclerosis (ALS), that currently have no proven treatments.



SBMA belongs to a group of neurodegenerative disorders, called polyglutamine diseases, that includes Huntington’s disease and spinocerebellar ataxias. Polyglutamine diseases are thought to arise because of a mutant protein that is misfolded and subsequently clumps together to form toxic aggregates that destroy cell function and cause disease. In SBMA, a mutated gene directs production of androgen receptors with an abnormal number of consecutive residues of the amino acid glutamine. Dr. Albert R. La Spada and colleagues from the University of Washington Medical Center in Seattle created transgenic mice containing the human androgen receptor carrying 100 glutamine repeats. The mice developed a gradually progressive limb weakness around mid-adulthood that was accompanied by motor neuron degeneration, strikingly similar to what is seen in human SBMA patients. The researchers determined that the abnormal androgen receptor interfered with production of a molecule called vascular endothelial growth factor (VEGF) that is important for the general health and survival of motor neurons. Interestingly, VEGF could rescue SBMA-like motor neurons grown in the laboratory.

The researchers conclude that VEGF may play a pivotal role in motor neuron degeneration. "Our findings in SBMA suggest that activation of the VEGF pathway may be one way that the motor neuron protects itself from harmful insults and stresses. Studies of ALS (amyotrophic lateral sclerosis) also point to the VEGF axis as critical for motor neuron health, so it is distinctly possible that all motor neuron diseases share interruption of the VEGF axis as part of their pathogenesis," explains Dr. La Spada. "If this is true, then it would have dramatic implications for treatment of motor neuron diseases."


Bryce L. Sopher, Patrick S. Thomas, Jr., Michelle A. LaFevre-Bernt, Ida E. Holm, Scott A. Wilke, Carol B. Ware, Lee-Way Jin, Randell T. Libby, Lisa M. Ellerby, and Albert R. La Spada: "Androgen Receptor YAC Transgenic Mice Recapitulate SBMA Motor Neuronopathy and Implicate VEGF164 in the Motor Neuron Degeneration"


Published in Neuron, Volume 41, Number 5, 4 March 2004, pages 687-699.

Heidi Hardman | EurekAlert!
Further information:
http://www.cell.com/

More articles from Life Sciences:

nachricht Dissolving protein traffic jam at the entrance of mitochondria
23.05.2019 | Albert-Ludwigs-Universität Freiburg im Breisgau

nachricht Producing tissue and organs through lithography
23.05.2019 | Goethe-Universität Frankfurt am Main

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: The geometry of an electron determined for the first time

Physicists at the University of Basel are able to show for the first time how a single electron looks in an artificial atom. A newly developed method enables them to show the probability of an electron being present in a space. This allows improved control of electron spins, which could serve as the smallest information unit in a future quantum computer. The experiments were published in Physical Review Letters and the related theory in Physical Review B.

The spin of an electron is a promising candidate for use as the smallest information unit (qubit) of a quantum computer. Controlling and switching this spin or...

Im Focus: Self-repairing batteries

UTokyo engineers develop a way to create high-capacity long-life batteries

Engineers at the University of Tokyo continually pioneer new ways to improve battery technology. Professor Atsuo Yamada and his team recently developed a...

Im Focus: Quantum Cloud Computing with Self-Check

With a quantum coprocessor in the cloud, physicists from Innsbruck, Austria, open the door to the simulation of previously unsolvable problems in chemistry, materials research or high-energy physics. The research groups led by Rainer Blatt and Peter Zoller report in the journal Nature how they simulated particle physics phenomena on 20 quantum bits and how the quantum simulator self-verified the result for the first time.

Many scientists are currently working on investigating how quantum advantage can be exploited on hardware already available today. Three years ago, physicists...

Im Focus: Accelerating quantum technologies with materials processing at the atomic scale

'Quantum technologies' utilise the unique phenomena of quantum superposition and entanglement to encode and process information, with potentially profound benefits to a wide range of information technologies from communications to sensing and computing.

However a major challenge in developing these technologies is that the quantum phenomena are very fragile, and only a handful of physical systems have been...

Im Focus: A step towards probabilistic computing

Working group led by physicist Professor Ulrich Nowak at the University of Konstanz, in collaboration with a team of physicists from Johannes Gutenberg University Mainz, demonstrates how skyrmions can be used for the computer concepts of the future

When it comes to performing a calculation destined to arrive at an exact result, humans are hopelessly inferior to the computer. In other areas, humans are...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

SEMANTiCS 2019 brings together industry leaders and data scientists in Karlsruhe

29.04.2019 | Event News

Revered mathematicians and computer scientists converge with 200 young researchers in Heidelberg!

17.04.2019 | Event News

First dust conference in the Central Asian part of the earth’s dust belt

15.04.2019 | Event News

 
Latest News

Plumbene, graphene's latest cousin, realized on the 'nano water cube'

23.05.2019 | Materials Sciences

New flatland material: Physicists obtain quasi-2D gold

23.05.2019 | Materials Sciences

New Boost for ToCoTronics

23.05.2019 | Physics and Astronomy

VideoLinks
Science & Research
Overview of more VideoLinks >>>