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:10/13/2017)... ... October 13, 2017 , ... ... process to promote standards of excellence for the field of eating disorders, announces ... 22 – 25, 2018 in Orlando, Florida at the Omni Resort at ChampionsGate. ...
(Date:10/13/2017)... ... 13, 2017 , ... Lori R. Somekh, founder of the Law Office ... of elder law and special needs planning attorneys. “Membership in ElderCounsel helps our office ... forum to network with elder law attorneys nationwide,” said Somekh. , ElderCounsel ...
(Date:10/13/2017)... ... , ... The Visiting Nurse Association (VNA) of Somerset Hills is proud to ... unique items from across the nation, this holiday-themed event will raise funds and awareness ... VNA. The boutique will be open Saturday, November 4 (10:00 a.m. – 5:00 ...
(Date:10/13/2017)... (PRWEB) , ... October 13, 2017 , ... Yisrayl Hawkins, ... week that explains one of the most popular and least understood books in the ... cryptic and puzzling descriptions that have baffled scholars for centuries. Many have tossed it ...
(Date:10/13/2017)... , ... October 13, 2017 , ... “The Journey: From ... every danger possible to save lost souls in the Philippines. “The Journey: From the ... is a dedicated teacher of the Bible. She has taught all ages and currently ...
Breaking Medicine News(10 mins):
(Date:10/2/2017)... , Oct. 2, 2017 Halo Labs announces the ... analysis system called the HORIZON at MIBio 2017 in ... subvisible and visible particulate matter in biopharmaceutical samples with unprecedented speed ... of the novel technique Backgrounded Membrane Imaging. ... The HORIZON subvisible particle analysis system ...
(Date:9/27/2017)... CAESAREA, Israel and NEW YORK , Sept. ... company with mobile health and big data solutions, today announced that its ... today. Please check your local TV listings for when The Dr. Oz ... ... ninth season this month. ...
(Date:9/22/2017)... -- AVACEN Medical (AVACEN) announced that its CE-Marked AVACEN ... with the widespread pain associated with fibromyalgia in the ... Essex, England commented, "I had difficulty ... sleep at all, tremendous pain, with every movement sending ... AVACEN 100] enough, how this has and is helping ...
Breaking Medicine Technology:
Cached News: