HOME >> MEDICINE >> NEWS
Mouse model of neuromuscular disease identifies key player in motor neuron death

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
'"/>

Contact: Heidi Hardman
hhardman@cell.com
617-397-2879
Cell Press
3-Mar-2004


Page: 1 2

Related medicine news :

1. Mouse model reveals potential way to reduce cardiac deaths in kidney patients
2. Mouse gene shows new mechanism behind cardiac infarction in man
3. Mouse model gives insight to human hair loss
4. Mouse study: Signal overload in Alzheimer brains
5. Mouse shows how Rituximab removes human B cells
6. Mouse study could aid vaccine designers
7. Mouse model mimics real-world plague infection
8. Mouse antibodies thwart SARS virus
9. Mouse study gives new view of anthrax toxin
10. Mouse studies reveal immune mechanism involved in bone loss caused by estrogen deficiency
11. Mouse provides clues about chronic pain

Post Your Comments:
*Name:
*Comment:
*Email:


(Date:6/24/2016)... ... ... Inc, makers of Topricin and MyPainAway Pain Relief Products, join The ‘Business for a Fair ... hour by 2020 and then adjusting it yearly to increase at the same rate as ... the wage floor does not erode again, and make future increases more predictable. , The ...
(Date:6/24/2016)... ... June 24, 2016 , ... Puradigm® & Innovative ... has initiated cultivation and processing operations at its production facility, and opened its ... Puradigm is the manufacturer of a complete system of proactive air and surface ...
(Date:6/24/2016)... (PRWEB) , ... June 24, 2016 , ... ... for human induced pluripotent stem (iPS) cells and other difficult to transfect cells, ... Cloning Medium. The PluriQ™ G9™ Gene Editing System is a complete ...
(Date:6/24/2016)... ... 2016 , ... Southern Illinois University School of Dental Medicine ... State Dental Society (ISDS) Foundation’s Mission of Mercy (MOM). They will host a ... , They expect to treat approximately 2,000 patients from Illinois, Missouri, ...
(Date:6/23/2016)... , ... June 23, 2016 , ... ... Supply Foundation, a not-for-profit organization responsible for clinical transfusion research programs and for ... for the Pathogen Reduction Evaluation and Predictive Analytical Rating Score (PREPAReS) study. ...
Breaking Medicine News(10 mins):
(Date:6/24/2016)... , June 24, 2016 VolitionRx ... of Dr. Edward Futcher to the ... effective June 23, 2016.Dr. Futcher was also appointed ... Governance Committees.  As a non-executive member of the ... and strategic counsel to VolitionRx in connection with ...
(Date:6/23/2016)... -- Research and Markets has announced the ... (Organic Chemical (Sugar, Petrochemical, Glycerin), Inorganic Chemical), Functionality (Filler, ... Global Forecast to 2021" report to their offering. ... pharmaceutical excipients market is projected to reach USD 8.1 ... the forecast period 2016 to 2021. ...
(Date:6/23/2016)... 23, 2016 Research and Markets has ... 52" report to their offering. ... creates a favourable commercial environment for MedImmune to enter. The ... that will serve to drive considerable growth for effective anti-influenza ... to cap sales considerably, but development is still in its ...
Breaking Medicine Technology:
Cached News: