Reduced activity-dependent protein levels in a mouse model of the fragile X premutation

Neurobiol Learn Mem. 2014 Mar:109:160-8. doi: 10.1016/j.nlm.2014.01.011. Epub 2014 Jan 23.

Abstract

Environmental enrichment results in increased levels of Fmrp in brain and increased dendritic complexity. The present experiment evaluated activity-dependent increases in Fmrp levels in the motor cortex in response to training on a skilled forelimb reaching task in the CGG KI mouse model of the fragile X premutation. Fmrp, Arc, and c-Fos protein levels were quantified by Western blot in the contralateral motor cortex of mice following training to reach for sucrose pellets with a non-preferred paw and compared to levels in the ipsilateral motor cortex. After training, all mice showed increases in Fmrp, Arc, and c-Fos protein levels in the contralateral compared to the ipsilateral hemisphere; however, the increase in CGG KI mice was less than wildtype mice. Increases in Fmrp and Arc proteins scaled with learning, whereas this relationship was not observed with the c-Fos levels. These data suggest the possibility that reduced levels of activity-dependent proteins associated with synaptic plasticity such as Fmrp and Arc may contribute to the neurocognitive phenotype reported in the CGG KI mice and the fragile X premutation.

Keywords: Arc; CGG KI mouse; Endophenotype; Fmrp; Fragile X premutation; Western blot; c-Fos.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Disease Models, Animal
  • Fragile X Mental Retardation Protein / genetics
  • Fragile X Mental Retardation Protein / metabolism*
  • Fragile X Syndrome / genetics
  • Fragile X Syndrome / metabolism*
  • Gene Knock-In Techniques
  • Male
  • Mice
  • Motor Activity / physiology*
  • Motor Cortex / metabolism*
  • Mutation

Substances

  • Fmr1 protein, mouse
  • Fragile X Mental Retardation Protein