UNO Researchers Discover Way to Delay Symptoms of Deadly Huntington's Disease

Researchers at the University of New Orleans have discovered a way to significantly delay the symptoms of Huntington's Disease in mice carrying the human Huntington's Disease gene. Huntington's Disease is an untreatable, incurable and fatal disease that causes certain nerve cells in the brain to waste away. The research is published in the online journal PLOS (Public Library of Science) ONE.

"I believe that these findings are important because they may lead to the development of the first treatment for this horrible disease," said Gerald LaHoste, associate professor of psychology.

For years, scientists have known that a mutated version of the gene known as huntingtin is present in people suffering from Huntington's Disease. UNO researchers, led by LaHoste, have identified a second gene that may be linked to the disease. Based on their findings, they believe that a class of cholesterol-lowering drugs, called statins, could greatly slow the symptoms of Huntington's Disease in humans. That research is ongoing at UNO.

The researchers studied mice that had the mutated human version of the huntingtin gene incorporated into their DNA. They discovered that the presence of another gene, called Rhes, is necessary for the symptoms of Huntington's Disease to appear. When the Rhes gene was inhibited, researchers observed a significant delay in the symptoms in the mice. Relative to the lifespan of these mice, the delay translates to about five years in humans.

"Mice carrying the huntingtin gene but not the Rhes gene showed no symptoms at 4 months of age, at which time those with the Rhes gene showed 50 percent impairment in motor function," LaHoste said.

Huntington's Disease is an inherited disease that affects one in 10,000 people of European descent. People are born with the defective gene, but symptoms usually don't appear until middle age. Early symptoms of Huntington's Disease may include uncontrolled movements, clumsiness or balance problems. Later, the disease can take away the ability to walk, talk or swallow. Most patients develop dementia and many display schizophrenia-like symptoms. Death occurs after 10 to 15 years of suffering. Children of people with Huntington's Disease have a 50-50 chance of acquiring it.

To read the paper visit: http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0053606