Protective pathway in brain seems to shed light on Huntington disease

Huntington’s disease (HD) is said to be a hereditary condition that may result in acute physical and mental deterioration, psychiatric issues and ultimately, death. Presently, there seems to be no treatments to decelerate or stop it.

People with this disease apparently do not exhibit symptoms until late in life. Stephen Ferguson and Fabiola Ribeiro of Robarts Research Institute at The University of Western Ontario supposedly recognized a defensive pathway in the brain that may clarify why HD symptoms take so long to materialize. The discoveries may result in potential new treatments for HD.

Apparently, the symptoms of Huntington’s disease are due to cell death in particular areas of the brain. Patients who suffer from HD are said to be born with a mutated version of the protein huntingtin (Htt), which is believed to cause these toxic effects. While scientists identify HD outcomes from this single, mutated protein, no one appears to know precisely what it does, why it may not cause symptoms until later in life, or why it destroys a particular group of brain cells, although Htt appears to be discovered in every single cell in the human body.

Ferguson and Ribeiro supposedly utilized a genetically-modified mouse model of HD to observe the consequences of mutated Htt on the brain.

Ferguson, director of the Molecular Brain Research Group at Robarts, and a professor in the Department of Physiology & Pharmacology at Western’s Schulich School of Medicine and Dentistry, commented, “We found there was some kind of compensation going on early in the life of these mice that was helping to protect them from the development of the disease. As they age, they lose this compensation and the associated protective effects, which could explain the late onset of the disease.”

Ferguson includes metabotropic glutamate receptors (mGluRs), which seems to be accountable for interaction between brain cells. It may play a significant function in these defensive consequences. By communicating with the mutant Htt protein, mGluRs apparently alters the way the brain seems to indicate in the initial stages of HD in an effort to compensate the disease, and save the brain from cell death. Consequently, mGluRs may provide a drug target for HD treatment.

The research was published in the Journal of Neuroscience.