Huntington's and a New Protein Found in the Brain

Rhes, the Protein in Connection With Huntington's Disease

Jun 7, 2009

Researchers may have found that the protein in Huntington's disease, may not be acting alone. New discoveries are just the beginning, hopefully leading to a cure.

If researchers are right about this discovery, it will help explain why some neurons in one specific part of the brain are susceptible to this disease. There is an article in Medical News Today about research being done to help people with Huntington's disease. In 1993, scientists found that a mutant form of the protein huntingtin is the cause of Huntington's disease. Although every cell in the body has this protein, it only turns deadly when it attacks one section of the brain cells, leading to Huntington's disease. That place in the brain is the corpus striatum. This part of the brain controls body movement. The reason why the mutant huntingtin kills these cells remains a mystery, but now that they know the cause, finding a cure is coming closer to reality. .

Rhes Attacks and Kills Cells in the Striatum

Researchers at Johns Hopkins University in Baltimore figured out why a mutated protein accumulated in cells all over the body in those diagnosed with Huntingtons disease, but not sure why it attacks and kills cells in the part of the brain, which controls movement. As reported in June 2009 in Science, the one tiny protein responsible is Rhes, (also known as Ras homolog enriched in striatum). Researchers believe this protein is responsible for goading huntingtin into killing the brain cells in the striatum.

Huntington's disease is a genetic defect passed from parent to child through an alteration of a normal gene. Over time, Huntington's disease causes uncontrolled movements, loss of intellectual function, emotional disturbance and death. So far, the effects of Huntington's disease are irreversible.

This Rhes protein is what they believe leads to Huntington's disease. Nancy Wexler, president of the Hereditary Disease Foundation and a Huntington's disease researcher at Columbia University, reports that if this can be confirmed, it could lead them down new avenues for developing a treatment for the fatal neurodegenerative disease.

Researchers have studied the role of individual proteins that cause or prevent disease, but few ever go beyond the molecular level to find out why neurodegenerative diseases attack the brain, or certain parts of it. Now that they know this gene destroys a particular part of the brain, they know there is potential to finding the right drug that will target it and prevent these symptoms from coming on, or can slow them down.

By continuing their studies using human, and mouse cells they discovered that the Rhes interacts with both healthy and mutant huntingtin protein,. But when it is with the mutant huntingtin, otherwise known as mHtt, it binds to it strongly.

Rhes, the Key to Huntington's Disease

According to Medical News Today there is a report of one study being done using human embryonic cells and brain cells from mice. Scientists added mutant and normal huntintin and Rhes, in a variety of different combinations. This experiment was conducted for a week’s time.

What they discovered was neither protein, when alone, changed the number of cells in each dish. It was only when huntingtin and Rhes were in the same cells that half of those cells died within 48 hours.

Perhaps with time, a cure will be gained from this new knowledge. Advances are being made and since the discovery of the Rhes, a major jump has been gained. Given time, Huntington's disease could be a health problem of the past

The copyright of the article Huntington's and a New Protein Found in the Brain in Healthcare Research is owned by Gail Delaney. Permission to republish Huntington's and a New Protein Found in the Brain in print or online must be granted by the author in writing.