Motor deficits on a ladder rung task in male and female adolescent and adult CGG knock-in mice

作者：Hunsaker Michael R^*; von Leden Ramona E; Ta Binh T; Goodrich Hunsaker Naomi J; Argue Gloria; Kim Kyoungmi; Willemsen Rob; Berman Robert F

来源：Behavioural Brain Research, 2011, 222(1): 117-121.

DOI：10.1016/j.bbr.2011.03.039

• Fragile X premutation
• Transgenic mice
• Motor function
• Endophenotype
• Neurodevelopmental disorder
• Neurodegenerative disorder
• FXTAS

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.

• 出版日期2011-9-12