Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation

Michael R. Hunsaker, Naomi J. Goodrich-Hunsaker, Rob Willemsen, Robert F Berman

Research output: Contribution to journalArticle

47 Scopus citations

Abstract

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 repeat lengths between 70 and 350 has been developed and used to characterize 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 information. To further characterize cognitive deficits in the fragile X premutation, temporal ordering in CGG knock-in (CGG KI) mice was evaluated. Female CGG KI mice were tested for their ability to remember the temporal order in which two objects were presented. The results demonstrate that at 48 weeks of age, female CGG KI mice with CGG repeat expansions between 150 and 200 CGG repeats performed more poorly on tests of temporal order than wildtype mice, whereas female CGG KI mice with between 80 and 100 CGG repeats performed similarly to wildtype mice. No mice had any difficulty in detecting the presence of a novel object. These data suggest female CGG KI mice show a CGG repeat length-sensitive deficit for temporal ordering.

Original languageEnglish (US)
Pages (from-to)263-268
Number of pages6
JournalBehavioural Brain Research
Volume213
Issue number2
DOIs
StatePublished - Dec 2010

Keywords

  • Fragile X premutation
  • Knock-in mice
  • Learning and memory
  • Novel object recognition
  • Pattern separation
  • Temporal processing

ASJC Scopus subject areas

  • Behavioral Neuroscience
  • Medicine(all)

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