19 June 2012

Study Hints Coenzyme Q10 (CoQ) Slows Progression of Huntington's Disease

How Huntington's Disease can be passed down
Huntington's disease (HD) is a disease that causes nerve cells in the brain to degenerate. These nerve cells called neurons when affected by HD causes uncontrolled movements, loss of intellectual faculties and emotional disturbance.

Huntington's disease is hereditary, passed from parent to child through a defective gene. A child has a 50% chance of inheriting the mutant gene from his or her parents. If inherited, the person is surely to manifest HD sooner or later. Otherwise, the child is safe and will not be able to pass down the disease to his/her own children.

People with HD manifest symptoms when they hit middle age. These include uncontrolled movements, loss of equilibrium, and clumsiness. As the disease progresses, the affected patient would have difficulty (or even unable) in talking, walking, or swallowing. Cognitive skills such as recognition are also affected.

There is no known cure for the disease.

What is Coenzyme Q10?

Coenzyme Q10 (CoQ or CoQ10) is a natural compound found in the body that helps convert food into energy. It is found in almost every cell (primarily in the mitochondria) in the body and is a powerful antioxidant.

Primary dietary sources of CoQ10 include oily fish (such as salmon and tuna), organ meats (such as liver), and whole grains. Most people get enough CoQ10 through a balanced diet.

It is an important compound used by cells to generate energy. 95% of the human body's energy is generated with the help of CoQ. Organs with the highest energy requirements such as the heart, liver, and kidney have the highest CoQ10 concentrations.

Study indicates promise in Huntington's treatment

A new study shows that the compound Coenzyme Q10 (CoQ) reduces oxidative damage, a key finding that hints at its potential to slow the progression of Huntington disease. The discovery, which appears in the inaugural issue of the Journal of Huntington's Disease, also points to a new biomarker that could be used to screen experimental treatments for this and other neurological disorders.

"This study supports the hypothesis that CoQ exerts antioxidant effects in patients with Huntington's disease and therefore is a treatment that warrants further study," says University of Rochester Medical Center neurologist Kevin M. Biglan, M.D., M.P.H., lead author of the study. "As importantly, it has provided us with a new method to evaluate the efficacy of potential new treatments."

Video: Huntington's Disease

Huntington's disease (HD) is a genetic, progressive neurodegenerative disorder that impacts movement, behavior, cognition, and generally results in death within 20 years of the disease's onset. While the precise causes and mechanism of the disease are not completely understood, scientists believe that one of the important triggers of the disease is a genetic "stutter" which produces abnormal protein deposits in brain cells. It is believed that these deposits – through a chain of molecular events – inhibit the cell's ability to meet its energy demands resulting in oxidative stress and, ultimately, cellular death.

Scientists had previously identified the correlation between a specific fragment of genetic code, called 8-hydroxy-2'-deoxyguanosine (80HdG) and the presence of oxidative stress in brain cells. 80HdG can be detected in a person's blood, meaning that it could serve as a convenient and accessible biomarker for the disease. Researchers have also been evaluating the compound Coenzyme Q10 as a possible treatment for HD because of its ability to support the function of mitochondria – the tiny power plants the provide cells with energy – and counter oxidative stress.

The study's authors evaluated a series of blood samples of 20 individuals with HD who had previously undergone treatment with CoQ in clinical trial titled Pre-2Care. While these studies showed that CoQ alleviated some symptoms of the disease, it was not known what impact – if any – the treatment had at the molecular level in the brain. Upon analysis, the authors found that 80HdG levels dropped by 20 percent in individuals who had been treated with CoQ.

CoQ is currently being evaluated in a Phase 3 clinical trial, which is the largest therapeutic clinical study to date for HD. The trial – called 2Care – is being run by the Huntington Study Group, an international networks or investigators.

"Identifying treatments that slow the progression or delay the onset of Huntington's disease is a major focus of the medical community," said Biglan. "This study demonstrates that 80HdG could be an ideal marker to identify the presence oxidative injury and whether or not treatment is having an impact."


University of Rochester Medical Center
Journal of Huntington's Disease
Huntington Study Group
Huntington's Disease
NINDS Huntington's Disease Information Page
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