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:1/21/2017)... ... January 21, 2017 , ... Salveo for life, a ... bringing its product to the United States as part of its presence to expand ... years, Alcovit aims to reduce the productions of nasty toxins as a result of ...
(Date:1/21/2017)... (PRWEB) , ... January 21, 2017 , ... ... Redwood Family Dermatology has recently joined their multi-specialty medical group. The ... range of cosmetic services. , “We’re excited to add this excellent dermatology practice ...
(Date:1/20/2017)... ... January 20, 2017 , ... A new partnership between Goodwill® ... no longer use or need, from clothes to couches to dressers and bicycles. Roadie ... take them to the nearest Goodwill donation center through February 28th. , “January ...
(Date:1/20/2017)... ... ... Lice Troopers, the lice removal company based in South Florida, has seen a ... , “It happens every year around this time,” says owner, Arie Harel. “Many ... the head-to-head gateway that lice need to spread.” , As children return to classrooms ...
(Date:1/20/2017)... ... January 20, 2017 , ... Vitamin ... alternative VW+ 002. The drinks have been produced in collaboration with Zlatan Ibrahimovic ... during your workout. , After a successful launch in Sweden last year, the ...
Breaking Medicine News(10 mins):
(Date:1/19/2017)... 19, 2017 Incretin Mimetics/GLP-1 Agonists, SNDRIs, Lipase ... The global anti-obesity drugs market is expected to ... the forecast period and CAGR of 38.7% in the second half ... a CAGR of 32.8% from 2016 to 2027. The market is ... $24,063 million in 2027. ...
(Date:1/19/2017)... -- Accuray Incorporated (Nasdaq: ARAY ) announced ... set the bar for excellence in customer satisfaction. The ... rating among radiation treatment delivery systems in the U.S., ... Briefing™. The most recent ratings trend also shows Accuray ... for 11 of the past 12 quarters. ...
(Date:1/19/2017)... LONDON , January 19, 2017 ... Grothey ; European Oncology & Haematology, 2016;12(Suppl 2):3-8; ... ... http://photos.prnewswire.com/prnh/20151014/276718LOGO) Published recently in ... the peer-reviewed journal from touchONCOLOGY, an article by ...
Breaking Medicine Technology:
Cached News: