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:
(Date:10/31/2014)... BCC Research ( http://www.bccresearch.com ) reveals in ... , the U.S. indoor air quality (IAQ) market is expected ... annual growth rate (CAGR) of 7% over the next five ... a CAGR of 7.4%. , Since 2012, continuing media ... outbreak of infectious diseases such as bird flu and the ...
(Date:10/31/2014)... Alpharetta, GA (PRWEB) October 31, 2014 ... that physicians see very little difference between diagnostic ... Howard, Director of Marketing for DigitalOne. “Differentiation ... more so in Radiology when you consider the ... Diagnostic Imaging of Salem will stand-out from ...
(Date:10/31/2014)... 31, 2014 Adults 65 and ... their Medicare coverage during the current open enrollment ... library for step-by-step guidance, the company announced today. ... on choosing the correct Medicare Advantage plan, understanding ... open enrollment. , “With just one month remaining ...
(Date:10/31/2014)... 2014 (HealthDay News) -- Carving pumpkins and trick-or-treating may ... children to emergency rooms in the United States every ... had the fifth highest number of ER visits involving ... data from the National Electronic Injury Surveillance System. ... head, noted the American Academy of Orthopaedic Surgeons and ...
(Date:10/31/2014)... Dennis Thompson ... News) -- A full-court press involving all public health ... to quell the current West African epidemic, a new ... the Ebola-infected dead in a hygienic way, immediately isolating ... and providing better protection for health care workers -- ...
Breaking Medicine News(10 mins):Health News:Global Market for U.S. Indoor Air Quality (IAQ) to Reach $11.4 Billion by 2019; Equipment Market Segment Surging at 7.4% CAGR 2Health News:Global Market for U.S. Indoor Air Quality (IAQ) to Reach $11.4 Billion by 2019; Equipment Market Segment Surging at 7.4% CAGR 3Health News:Diagnostic Imaging of Salem Expects Growth Partnering with DigitalOne 2Health News:Halloween at the ER Is No Treat 2Health News:4 Steps Could Quell Ebola in West Africa, Researchers Say 2Health News:4 Steps Could Quell Ebola in West Africa, Researchers Say 3
(Date:10/31/2014)... 2014  Semler Scientific, Inc. (Nasdaq: ... company that develops patented products that assist healthcare ... today reported financial results for the third quarter ... "In the third quarter of 2014, ... M.D., chief executive officer of Semler.  "We moved ...
(Date:10/31/2014)... , Oct. 31, 2014  Australian biopharmaceutical company Specialised ... (nanoparticle albumin-bound paclitaxel) in combination with gemcitabine, will ... patients with metastatic pancreatic cancer from 1 November. ... cancer is the 5 th most common cause ... all cancer deaths, with the lowest 5-year survival of ...
(Date:10/31/2014)... -- Investor-Edge has initiated coverage on the ... Isis Pharmaceuticals Inc. (NASDAQ: ISIS ), Halozyme ... Inc. (NASDAQ: ACAD ), and Synta Pharmaceuticals ... can be accessed at: http://investor-edge.com/register . ... at 4,566.14, up 0.37%, the Dow Jones Industrial Average ...
Breaking Medicine Technology:Semler Reports Third Quarter 2014 Financial Results 2Semler Reports Third Quarter 2014 Financial Results 3Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 2Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 3Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 4Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 5Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 6Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 7Specialised Therapeutics Australia Announces Breakthrough Cancer Drug Abraxane(R) to be PBS Listed for Metastatic Pancreatic Cancer 8Momentum Indicators for Biotech Stocks -- Nanosphere, Isis Pharma, Halozyme Therapeutics, ACADIA Pharma, and Synta Pharma 2Momentum Indicators for Biotech Stocks -- Nanosphere, Isis Pharma, Halozyme Therapeutics, ACADIA Pharma, and Synta Pharma 3Momentum Indicators for Biotech Stocks -- Nanosphere, Isis Pharma, Halozyme Therapeutics, ACADIA Pharma, and Synta Pharma 4Momentum Indicators for Biotech Stocks -- Nanosphere, Isis Pharma, Halozyme Therapeutics, ACADIA Pharma, and Synta Pharma 5
Cached News: