Genes and Huntington's Disease

Genetic counseling is a wonderful tool to learn about your genes and the genes you are passing on to your children. Some fatal genetic disorders, such as Huntington’s disease, which causes some brain cells to waste away, may also be transferred genetically.

If one parent has Huntington's disease, their child has a 50 percent chance of receiving the gene. The disease symptoms include uncontrolled movements, psychiatric disturbance, gradual dementia and eventual death.

Seek a genetic counselor if you have Huntington's disease questions.

Huntington's disease is usually adult-onset when symptoms begin and there is currently no therapy available which can slow the disease's progression.

Laboratory research at the University of Texas Southwestern Medical School has contributed to growing scientific evidence that suggests differences in neuronal calcium signaling play a key role in Huntington’s disease development and progression. The current study shows that the quinoline compounds – supplied by EnVivo, protects certain brain cells.

Senior author Ilya Bezprozvanny, PhD and professor of physiology at University of Texas Southwestern Medical School reports that their research has uncovered a new therapeutic target for Huntington’s disease treatment and hopefully other neurodegenerative diseases. Now, with this new series of compounds researchers have a tool to study how Huntington’s disease progresses.

Bezprozvanny's lab found that quinazoline-derived compounds block the store-operated calcium entry signaling pathway, which never been implicated before in Huntington nerve cells, but could be a therapeutic target in the disease.

This study is published in the June 23, 2011 issue of Chemistry and Biology.