Navigation Links
Huntington's disease breakthrough equals hope for patients
Date:2/22/2011

A huge leap forward in understanding Huntington's disease may give patients hope for a cure.

Laboratory tests on skin cells and post-mortem brain tissue of Huntington's disease patients determined that an overactive protein triggers a chain reaction that causes brain nerve cells to die. Toning down the activity of that protein, known as DRP1, prevented the chain reaction and kept those cells alive, according to the research team led by University of Central Florida Professor Ella Bossy-Wetzel.

Huntington's is an inherited, incurable neurodegenerative disease affecting 35,000 people annually. The disease gradually kills nerve cells in the brain, stripping away a person's physical abilities and causing hallucinations, antisocial behavior and paranoia.

People diagnosed with the disease usually die 15 to 20 years from the onset of symptoms, and there is an increased rate of suicide among those struggling with the disease.

"The next step will be to test the DRP1 function in animals and patients to see whether the protein also protects the brain," Bossy-Wetzel said. "This could be done before the onset of disease in patients who have the mutant Huntington gene, but have no neurological symptoms. The hope is that we might be able to delay the onset of disease by improving the energy metabolism of the brain."

The research findings were published online this week in the journal Nature Medicine, and they will be featured in the cover story of the March edition.

Until now, little has been known about how Huntington's works. Scientists knew that people with the mutant Huntington gene develop the disease. They also knew that a cell's powerhouse mitochondria, which turn food into energy was somehow involved. But until Bossy-Wetzel's team completed its work, little else was known.

"Mitochondria require balanced cycles of division and fusion to maintain their ability to produce energy," Bossy-Wetzel said. "The protein DRP1 is needed for mitochondrial division. We found that in Huntington's disease, DRP1 becomes overactive and causes too much mitochondrial division without balancing fusion."

That production error causes the brain's nerve cells to die. The UCF team toned down the activity of DRP1, which restored a normal balance of mitochondrial division and fusion and improved the energy metabolism and survival of neurons.

Other scientists in the field say the discovery is an important step toward eventually finding a cure.

"It is an outstanding piece of work, which further implicates mitochondrial dysfunction in the pathogenesis of Huntington's disease," said Flint Beal, a professor of neurology and neuroscience at the Weill Medical College of Cornell University who specializes in the disease and is a practicing physician. "It opens new therapeutic targets for therapies aimed at disease modification."


'/>"/>

Contact: Zenaida Gonzalez Kotala
zenaida.kotala@ucf.edu
407-823-6120
University of Central Florida
Source:Eurekalert  

Related medicine news :

1. Early life stress may predict cardiovascular disease
2. Study finds racial gaps continue in heart disease awareness
3. Bowel disease link to blood clots
4. Womens Heart Disease Awareness Still Lacking
5. American Heart Association Rapid Access Journal Report: Study Finds Racial Gaps Continue in Heart Disease Awareness, Low Knowledge of Heart Attack Warning Signs Among Women
6. New Study Uses Adult Stem Cells in Effort to Save Limbs of Patients with Peripheral Arterial Disease
7. Patients with Lethal Lung Disease Finally Receive Recognition by Social Security Administration
8. Alzheimers Foundation of America Applauds Social Security for Speeding Disability Benefits for Early-Onset Alzheimers Disease
9. New American Heart Association Survey Finds Heart Disease and Stroke Patients Face Significant Barriers in Obtaining Quality, Affordable Care
10. Hypnosis can relieve symptoms in children with respiratory diseases
11. IU research team discovers TB disease mechanism and molecule to block it
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Huntington's disease breakthrough equals hope for patients
(Date:10/13/2017)... ... October 13, 2017 , ... The Visiting Nurse Association (VNA) ... a collection of specialty vendors and unique items from across the nation, this holiday-themed ... and wellness services offered by the VNA. The boutique will be open Saturday, ...
(Date:10/13/2017)... Milford, NJ (PRWEB) , ... October 13, 2017 , ... ... weekend at scenic Alexandria Park in Milford, NJ. This free event, sponsored by ... and physical activity. The fun run is geared towards children of all ages; ...
(Date:10/13/2017)... ... 13, 2017 , ... Talented host, actor Rob Lowe, is ... a new episode of "Success Files," which is an award-winning educational program broadcasted ... each subject in-depth with passion and integrity. , Sciatica occurs when the sciatic ...
(Date:10/13/2017)... ... October 13, 2017 , ... “The Journey: From the ... danger possible to save lost souls in the Philippines. “The Journey: From the Mountains ... a dedicated teacher of the Bible. She has taught all ages and currently teaches ...
(Date:10/12/2017)... Del. (PRWEB) , ... October 12, 2017 , ... ... and advisory services for healthcare compliance program management, will showcase a range of ... National Association for Assisted Living (NCAL) Convention and Expo to be held October ...
Breaking Medicine News(10 mins):
(Date:9/27/2017)...  Commended for their devotion to personalized service, SMP Pharmacy ... one in the South Florida Business Journal,s 50 Fastest-Growing Companies, ... list, the national specialty pharmacy has found its niche.  To ... soon be honored by SFBJ as the 2017 Power Leader ... to receive his award in October, Bardisa said of the ...
(Date:9/25/2017)... , Sept. 25, 2017  EpiVax, Inc., a ... design, and immune-engineering today announced the launch of ... development of personalized therapeutic cancer vaccines. EpiVax has ... exclusive access to enabling technologies to the new ... will lead EpiVax Oncology as Chief Executive Officer. ...
(Date:9/22/2017)... 2017 AVACEN Medical (AVACEN) announced that its ... helping those with the widespread pain associated with fibromyalgia ... in Essex, England commented, "I ... experiencing no sleep at all, tremendous pain, with every ... recommend [the AVACEN 100] enough, how this has and ...
Breaking Medicine Technology: