Huntington's Disease

Huntington's Disease

Huntington's Disease is an inherited and fatal neurodegenerative disorder that affects about one in 10,000 Canadians, for which there is presently no cure. Called "one of the worst afflictions known to man", the relentless symptoms of this disease often begins at around age 40 and progresses until death. While symptoms vary individually, common traits include loss of motor control, slurred speech, and severe disturbances in cognition and emotional well-being.

Our Research

CMMT researchers have made tremendous strides in finding new ways to fight this disease. In 1999, CMMT researcher Dr. Hayden and his laboratory was the first to develop a mouse model for Huntington's Disease, which displays the behaviours that correspond to the human version of the disease. With over a decade of research dedicated to the illness and numerous discoveries, CMMT has truly become a leader in Huntington's Disease research in Canada, firmly committed to finding a cure.

One major breakthrough that could pave the way towards a cure for Huntington's Disease was discovered here at CMMT, when the laboratories of Drs. Hayden and Leavitt provided compelling evidence that the caspase-6 cleavage of the diseased, mutant Huntingtin protein is crucial in the pathogenesis of Huntington's Disease.

Mice expressing mutant Huntingtin that resists caspase-6 cleavage maintain normal neuronal function and do not develop cognitive or neurological abnormalities or any evidence of neurodegeneration. This represents the first intervention in any animal model for Huntington's Disease to prevent motor, cognitive and neuropathological features of the illness, and could lay the groundwork for an effective approach to therapy for the disease.

HD Clinic

We work very closely with the University of British Columbia Huntington's Disease Medical Clinic to offer predictive testing, a variety of support services and the opportunity to partake in a number of studies and drug trials. Please visit the Huntington's Disease Medical Clinic page to learn more.

Presentations

Jeff Carroll gave a talk at the Huntington's Disease Society of America Convention in June 2010. Please visit the HDSA website for a video of the presentation - "Empowering HD Families."

HD Resources

  • Huntington disease worldwide prevalence resource, a compilation of over a hundred published reports on the prevalence of HD in populations from Australia to Zimbabwe.

  • You can find more information on HD predictive testing at a newly launched website. This website contains more information for those considering predictive testing for HD in terms of the testing process, making the decision to be tested, interpreting results and coping with results.

Publications

Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G, Pearson J, Shehadeh J, Bertram L, Murphy Z, Warby SC, Doty CN, Roy S, Wellington CL, Leavitt BR, Raymond LA, Nicholson DW, Hayden MR. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 125(6):1179-91. (2006) PMID 16777606

Van Raamsdonk JM, Pearson J, Rogers DA, Bissada N, Vogl AW, Hayden MR, Leavitt BR. Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 14(10):1379-92. (2005) PMID 15829505