Coenzyme Q10 study indicates promise in Huntington's treatment

June 18, 2012

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 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."

Huntington's disease (HD) is a genetic, progressive 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 "" which produces deposits in . 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 for the disease. Researchers have also been evaluating the compound 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."

Explore further: Clinical trial examines antioxidant effects for Alzheimer's disease on cerebrospinal fluid biomarkers

More information: “Polyglutamine Expanded Huntingtin Dramatically Alters the Genome-Wide Binding of HSF-1,” by L. Riva, M. Koeva, F. Yildirim, et al. Journal of Huntington's Disease, Volume 1/Issue 1 (June 2012), DOI 10.3233/JHD-2012-120020

Related Stories

Clinical trial examines antioxidant effects for Alzheimer's disease on cerebrospinal fluid biomarkers

March 19, 2012
An antioxidant combination of vitamin E, vitamin C and α-lipoic acid (E/C/ALA) was not associated with changes in some cerebrospinal fluid biomarkers related to Alzheimer disease in a randomized controlled trial, according ...

Compound may provide drug therapy approach for Huntington's disease

June 23, 2011
UT Southwestern Medical Center researchers have identified compounds that appear to inhibit a signaling pathway in Huntington's disease, a finding that may eventually lead to a potential drug therapy to help slow the progression ...

Study finds transcriptional biomarker for Huntington's disease

October 3, 2011
Huntington's disease, a devastating genetic disorder that causes degeneration of nerve cells in the brain, affects more than 15,000 Americans, and at least 150,000 are at risk of developing the disease. There is no known ...

Reach2HD, a Phase II study in Huntington's disease, launched

June 7, 2012
The Huntington Study Group (HSG), under the leadership of Ray Dorsey, M.D. with Johns Hopkins Medical and Diana Rosas, M.D. with Massachusetts General Hospital, is conducting a clinical trial in Huntington's disease (HD) ...

Recommended for you

Mechanism explains how seizures may lead to memory loss

October 16, 2017
Although it's been clear that seizures are linked to memory loss and other cognitive deficits in patients with Alzheimer's disease, how this happens has been puzzling. In a study published in the journal Nature Medicine, ...

Study shows people find well-being more so from special places than from mementoes

October 16, 2017
(Medical Xpress)—A team of researchers at the University of Surrey has found that people experience a feeling of well-being when thinking about or visiting a place that holds special meaning to them. They also found that ...

New study describes how dopamine tells you it isn't worth the wait

October 16, 2017
How do we know if it was worth the wait in line to get a meal at the new restaurant in town? To do this our brain must be able to signal how good the meal tastes and associate this feeling with the restaurant. This is done ...

Neuroscientists identify genetic changes in microglia in a mouse model of neurodegeneration and Alzheimer's disease

October 13, 2017
Microglia, immune cells that act as the central nervous system's damage sensors, have recently been implicated in Alzheimer's disease.

Restless legs syndrome study identifies 13 new genetic risk variants

October 13, 2017
A new study into the genetics underlying restless legs syndrome has identified 13 previously-unknown genetic risk variants, while helping inform potential new treatment options for the condition.

Blueberries may improve attention in children following double-blind trial

October 13, 2017
Primary school children could show better attention by consuming flavonoid-rich blueberries, following a study conducted by the University of Reading.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.