A Canadian research team has identified an early-life protective compensation in brain cells that have the Huntingtin gene mutation that may lead to drug treatments for Huntington's disease (HD).
Stephen Ferguson and Fabiola Ribeiro of Robarts Research Institute at The University of Western Ontario wrote that they were able to identfy a protective mechanism in brain cells that might explain why HD symptoms take so long to appear and lead to new treatments. Their findings are published in The Journal of Neuroscience.

Huntington's disease (HD) is a debilitating, herediary disease that brings on gradual, relentless brain deterioration later in life, and eventually results in death. Scientists are seeking drug treatments that can slow or stop the progress of HD, a dominant genetic disease that is inherited by 50 percent of children with an affected parent.

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According to the Huntington's Outreach Project for Education (HOPES) of Standford University, the cell death in particular regions of the brain that bring on HD symptoms are thought be be caused by a mutated version of the protein huntingtin (Htt). Still, scientists don't know exactly what Htt does, or why the symptoms it causes are delayed until middle-age, or why it destroys only certain brain cells, when it is found in all the body's cells.

Ferguson and Ribeiro studied genetically-modified mice with the Htt gene to look at its effects on brain cells.

Furguson stated that they found a compensation involving metabotropic glutamate receptors (mGluRs) happening early in life that protected against the onset of the disease. He explained that mGluRs facilitate communication among brain cells and interact with the mutant Htt protein to change the way the brain signals in the early stages of HD to offset the condition and prevent cell death, and could offer a drug target for HD treatment.

Image: Coronal FSPGR through the brain at the level of the caudate nuclei demonstrating marked reduced volume in keeping with the patient's known diagnosis of Huntington disease. Image from [http://radiopaedia.org Radiopaedi), via Wikimedia Commons