There is very little known about Huntington’s disease (HD), a fatal neurodegenerative disease leading to total physical and mental decline that affects 30,000 Americans today.
Researchers have been developing transgenic mouse models to mimic human HD. A new model, the zQ175 knock-in, developed by Menalled et al. (2012) appears to more closely mimic human HD progression in the zQ175 KI than previous mouse models.
A recently published paper (co-authored by MBF’s President, Jack Glaser, and Staff Scientist Dr. Susan Hendricks) confirms the behavioral phenotypes reported by Menalled, and extends the characterization to include brain volumetry, striatal metabolite concentration, and early neurophysiological changes. For this study, MBF Labs used Stereo Investigator to quantify neural loss and regional volumetric changes. These changes validate the similarities between HD progression in humans and HD-like progression in zQ175 KI mice.
With this new mouse model, researchers might be able to considerably improve their understanding of the disease to find ways to temper HD progression.
Read the full paper “Characterization of Neurophysiological and Behavioral Changes, MRI Brain Volumetry and 1H MRS in zQ175 Knock-In Mouse Model of Huntington’s Disease” on PLOS.
Heikkinen T, Lehtimäki K, Vartiainen N, Puoliväli J, Hendricks SJ, et al. (2012). Characterization of Neurophysiological and Behavioral Changes, MRI Brain Volumetry and 1H MRS in zQ175 Knock-In Mouse Model of Huntington’s Disease. PLoS ONE 7(12): e50717. doi:10.1371/journal.pone.0050717
Menalled LB, Kudwa AE, Miller S, Fitzpatrick J, Watson-Johnson J, et al. (2012) Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175. PLoS ONE 7(12): e49838. doi:10.1371/journal.pone.0049838