Assistant Professor of Biology Elise Kikis, along with her students Amy Lee C’16, Paul Sands C17, and Hailey Ung, C’19, recently published a research article titled “A New Caenorhabditis elegans Model of Human Huntingtin 513 Aggregation and Toxicity in Body Wall Muscles” in the journal PLoS ONE. Mutant forms of the Huntingtin protein cause the progressive neurodegenerative disorder known as Huntington’s Disease.
Huntington’s Disease was first described in 1872 by the physician George Huntington. He was struck by the decline in motor skills and coordination that starts at around 40 years of age and gets progressively worse, ultimately culminating in early death.
Dr. Kikis and her team genetically engineered nematodes known as C. elegans to express a disease-causing form of human Huntingtin. The resultant animals are subject to the toxic effects of the protein, resulting in motility defects and shortened lifespan. Since they fluorescently labeled the huntingtin protein, they were able to monitor in real time how it aggregates and how aggregation changes during the course of aging. They expect their animals to serve as a useful disease model, especially for the study of how the huntingtin protein affects muscle cell function.