Publication Date

Spring 4-2023


School of Health Sciences


Biology: Biomedical Sciences


Huntington, genetic disease, tetrabenazine, deutetrabenazine


Nervous System Diseases


Huntington’s disease (HD) is a rare, incurable, inherited neurological disease that causes the progressive breakdown of nerve cells in the brain. The mutant huntingtin gene contains 40 to 80 repeats of a CAG trinucleotide sequence. The pathogenesis of HD is multifaceted and includes pathways related to glutamine aggregation, mitochondrial and oxidative stress, mitochondrial dysfunction, and DNA damage. Currently two drugs, tetrabenazine and deutetrabenazine, are FDA approved for the treatment of HD. Otherwise, medications given to HD patients include general antipsychotics, antidepressants, and mood-stabilizer drugs. Other treatment methods include speech and physical therapy. This research provides an understanding of the various maladapted HD pathways, as well as a comparison of the current HD drug therapies and other methods of treatment.