Grant: CIHR Fellowship

Huntington disease (HD) is an inherited, fatal, neurodegenerative disease characterised by loss of coordinated movement, dementia and psychiatric disturbances. Death usually occurs in 15-20 years after onset of symptoms. It is caused by a mutation of a protein called Huntingtin that causes the protein to build up and inhibit essential cellular functions that leads to neuronal cell death and eventually neurodegeneration. Proper localization of proteins within a cell is essential to their function, activities and for overall cell viability. Improper localization can lead to malfunctioning of proteins and in some cases cell death or cancer. The addition of lipid to specific proteins is one system the cell employs to regulate this process. A form of lipid modification known as myristoylation is essential for some proteins to re-localise in the cell. I recently identified a region of Huntingtin that undergoes this modification. It appears to be essential for maintaining healthy cell balance. I intend to study the role of this modification in the progression of Huntington's disease using a well established mouse model designed in the Hayden laboratory. This modification is a novel site of regulation of Huntingtin protein and may serve as an ideal target for therapeutic treatment of Huntington's disease.