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:8/16/2017)... ... August 16, 2017 , ... Any Lab Test ... The company ranked #4429 on the newly released, 36th annual Inc. 5000 , ... unique look at the most successful companies within the American economy’s most dynamic segment ...
(Date:8/16/2017)... Georgia (PRWEB) , ... August 16, 2017 , ... ... in Atlanta, opened their doors. They celebrate 30 years in business this year, ... create an up-to-date, inviting, tranquil space to serve their patients. , It ...
(Date:8/16/2017)... (PRWEB) , ... August 16, 2017 , ... ... practice partnerships through affiliations and de novo development, today announced Cumberland Skin ... , Cumberland Skin Surgery and Dermatology, with offices in both Hermitage and Lebanon, ...
(Date:8/16/2017)... ... ... Maury Regional Health has announced a large-scale adoption of AccuVein vein visualization ... Regional Medical Center is making vein visualization part of their standard of care. , ... importantly, helps our staff members locate a vein that will provide good access to ...
(Date:8/16/2017)... ... 2017 , ... Paul Vitenas, MD, FACS , is honored to announce ... list identifies the nation’s top physicians, in a variety of specialties. This marks the ... of Castle Connolly’s coveted ranking. , Castle Connolly is the nation’s trusted provider of ...
Breaking Medicine News(10 mins):
(Date:7/28/2017)... CHICAGO, July 28, 2017 Hill-Rom Holdings, Inc. ... fiscal third quarter ended June 30, 2017, and updated ... For the fiscal third quarter, ... to $0.68 per diluted share in the prior-year period. ... of $0.39 per diluted share primarily related to the ...
(Date:7/27/2017)... -- West Pharmaceutical Services, Inc. (NYSE: WST ) ... and updated financial guidance for the full-year 2017. ... Reported net sales of $397.6 million, a ... sales at constant currency (organic) grew by 3.9%. ... to $0.60 in the prior-year quarter. Second-quarter 2017 adjusted ...
(Date:7/26/2017)... , July 26, 2017 Sancilio Pharmaceuticals ... of our clinical trial evaluating Altemia TM , an ... (SCA) and Sickle Cell Disease (SCD). The SCOT Trial, ... the efficacy and safety of Altemia TM in ... conducted under US IND 125274. ...
Breaking Medicine Technology:
Cached News: