The fragile X premutation is a tandem CGG trinucleotide repeat expansion on the FMR1 gene between 55 and 200 repeats in length. A CGG knock-in (CGG KI) mouse with CGG trinucleotide repeat lengths between 70 and 350 has been developed and used to model the histopathology and cognitive deficits reported in carriers of the fragile X premutation. Previous studies have shown that CGG KI mice show progressive deficits in processing spatial and temporal information. To characterize the motor deficits associated with the fragile X premutation, male and female CGG KI mice ranging from 2 to 16 months of age with trinucleotide repeats ranging from 72 to 240 CGG in length were tested for their ability to perform a skilled ladder rung walking test. The results demonstrate that both male and female CGG KI mice showed a greater number of foot slips as a function of increased CGG repeat length, independent of the age of the animal or general activity level.

Additional Metadata
Keywords Endophenotype, FXTAS, Fragile X premutation, Motor function, Neurodegenerative disorder, Neurodevelopmental disorder, Transgenic mice
Persistent URL dx.doi.org/10.1016/j.bbr.2011.03.039, hdl.handle.net/1765/33625
Citation
Hunsaker, M.R., von Leden, R.E., Ta, B.T., Goodrich-Hunsaker, N.J., Arque, G., Kim, K., … Berman, R.F.. (2011). Motor deficits on a ladder rung task in male and female adolescent and adult CGG knock-in mice. Behavioural Brain Research, 222(1), 117–121. doi:10.1016/j.bbr.2011.03.039