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:4/28/2017)... New York, NY (PRWEB) , ... April 28, ... ... Shiel Medical Laboratory to expanded distribution of the GlycoMark test throughout the Northeast ... indicates recent hyperglycemia in patients with diabetes. The GlycoMark test provides a clinically ...
(Date:4/28/2017)... ... April 28, 2017 , ... Ushio America proudly ... offer an instant energy-saving solution for F32T8 fluorescent lamps on most instant-start and ... These 50,000 hour rated lamps utilize the existing electronic ballast, saving labor and ...
(Date:4/28/2017)... ... April 28, 2017 , ... Datta Endoscopic Back Surgery and ... revolutionary endoscopic practice under Dr. Datta. Patel is a Board Certified Anesthesiologist, with ... revolutionary eDiscSculpt Technique created and used by Datta Endoscopic is unlike traditional back ...
(Date:4/28/2017)... (PRWEB) , ... April 28, 2017 , ... The Texas ... in recently to the labor and delivery team at Women’s Hospital at Renaissance in ... who give birth at the hospital and decide to donate. , “Women’s Hospital ...
(Date:4/28/2017)... , ... April 28, 2017 , ... ... hERG liability could substantially improve drug safety and minimize the cost of development. ... validating ion channel inhibition using cell lines and for cardiac toxicity using induced ...
Breaking Medicine News(10 mins):
(Date:4/20/2017)... April 20, 2017   ZappRx, Inc ., a digital ... prescribing process, today announced it closed $25 million in Series ... capital firm based in Seattle that ... Partners . The Series B round included participation from ... 2014, and GV (formerly Google Ventures). As part ...
(Date:4/20/2017)... -- Eyevensys, a private biotechnology company developing ... technology that enables the safe, local, sustained production of ... range of ophthalmic diseases, announces it has received approval ... (MHRA) to advance its technology into clinical development. ... The EyeCET platform ...
(Date:4/19/2017)... - CRH Medical Corporation (TSX: CRH) (NYSE MKT: CRHM) (the "Company"), announces that ... Conference 2017 at the Sheraton Hotel in Toronto, Ontario ... of the Company is scheduled to present on Tuesday, May 2 ... the Chairman of the Board, Tony Holler will also ... For more ...
Breaking Medicine Technology: