Navigation Links
Huntington's disease breakthrough equals hope for patients

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
University of Central Florida

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:
Related Image:
Huntington's disease breakthrough equals hope for patients
(Date:6/27/2016)... ... June 27, 2016 , ... ... health professionals, announced today its affiliation with Tennessee Counseling Association. This ... the network of the Tennessee Counseling Association, adding exclusive benefits and promotional offers. ...
(Date:6/26/2016)... ... June 27, 2016 , ... Quality metrics are proliferating in cancer ... they remain in the eye of the beholder, according to experts who offered insights ... American Journal of Managed Care. For the full issue, click here . , ...
(Date:6/26/2016)... ... ... Kasmer, a legally blind and certified personal trainer is helping to develop a weight loss ... plans to fix the two major problems leading the fitness industry today:, ... , They don’t eliminate all the reasons people quit their exercise program ...
(Date:6/25/2016)... FL (PRWEB) , ... June 25, 2016 , ... The temporary closing of Bruton Memorial ... Plant City Observer , brings up a new, often overlooked aspect of head lice: the ... closing for fumigation is not a common occurrence, but a necessary one in the event ...
(Date:6/25/2016)... City, Oklahoma (PRWEB) , ... June 25, 2016 ... ... helping both athletes and non-athletes recover from injury. Recently, he has implemented orthobiologic ... the Oklahoma City area —Johnson is one of the first doctors to perform ...
Breaking Medicine News(10 mins):
(Date:6/24/2016)... , June 24, 2016 ... Market by Type (Standard Pen Needles, Safety Pen Needles), ... (Insulin, GLP-1, Growth Hormone), Mode of Purchase (Retail, Non-Retail) ... MarketsandMarkets, This report studies the market for the forecast ... to reach USD 2.81 Billion by 2021 from USD ...
(Date:6/23/2016)... , June 23, 2016  MedSource announced today ... its e-clinical software solution of choice.  This latest ... possible value to their clients by offering a ... preferred relationship establishes nowEDC as the EDC platform ... MedSource,s full-service clients.  "nowEDC has long been a ...
(Date:6/23/2016)... , June 23, 2016  In a startling report released today, ... their residents by lacking a comprehensive, proven plan to eliminate prescription ... definitive ranking of how states are tackling the worst drug crisis ... four states – Kentucky , New ... Vermont . Of the 28 failing states, three – ...
Breaking Medicine Technology: