Spatiotemporal processing deficits in female CGG KI mice modeling the fragile X premutation

Rachel M. Borthwell, Michael R. Hunsaker, Rob Willemsen, Robert F Berman

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

The fragile X premutation is a tandem CGG trinucleotide repeat expansion in the fragile X mental retardation 1 (FMR1) gene between 55 and 200 repeats in length. A CGG knock-in (CGG KI) mouse has been developed that models the neuropathology and cognitive deficits reported in fragile X premutation carriers. It has been suggested that carriers of the premutation demonstrate a spatiotemporal hypergranularity, or reduced resolution of spatial and temporal processing. A temporal ordering of spatial locations task was used to evaluate the ability of CGG KI mice to process temporal and spatial information with either high or low levels of spatial interference. The results indicate that CGG KI mice showed difficulty performing a spatial novelty detection task when there were high levels of spatial interference, but were able to perform the novelty detection task when there was low spatial interference. These data suggest that CGG KI mice show reduced spatial and temporal resolution that are modulated by the dosage of the Fmr1 gene mutation, such that when behavioral tasks require mice to overcome high levels of either spatial or temporal interference, the CGG KI mice perform increasingly poorly as the CGG repeat length increases.

Original languageEnglish (US)
Pages (from-to)29-34
Number of pages6
JournalBehavioural Brain Research
Volume233
Issue number1
DOIs
StatePublished - Jul 15 2012

Fingerprint

Trinucleotide Repeat Expansion
Aptitude
Gene Dosage
Intellectual Disability
Mutation
Genes
Neuropathology
Spatial Processing

Keywords

  • CGG KI mouse
  • Fragile X premutation
  • Pattern separation
  • Spatiotemporal processing
  • Temporal order

ASJC Scopus subject areas

  • Behavioral Neuroscience

Cite this

Spatiotemporal processing deficits in female CGG KI mice modeling the fragile X premutation. / Borthwell, Rachel M.; Hunsaker, Michael R.; Willemsen, Rob; Berman, Robert F.

In: Behavioural Brain Research, Vol. 233, No. 1, 15.07.2012, p. 29-34.

Research output: Contribution to journalArticle

Borthwell, Rachel M. ; Hunsaker, Michael R. ; Willemsen, Rob ; Berman, Robert F. / Spatiotemporal processing deficits in female CGG KI mice modeling the fragile X premutation. In: Behavioural Brain Research. 2012 ; Vol. 233, No. 1. pp. 29-34.
@article{e4e4b5a2e20b45eb9fa2160a36f7a6f5,
title = "Spatiotemporal processing deficits in female CGG KI mice modeling the fragile X premutation",
abstract = "The fragile X premutation is a tandem CGG trinucleotide repeat expansion in the fragile X mental retardation 1 (FMR1) gene between 55 and 200 repeats in length. A CGG knock-in (CGG KI) mouse has been developed that models the neuropathology and cognitive deficits reported in fragile X premutation carriers. It has been suggested that carriers of the premutation demonstrate a spatiotemporal hypergranularity, or reduced resolution of spatial and temporal processing. A temporal ordering of spatial locations task was used to evaluate the ability of CGG KI mice to process temporal and spatial information with either high or low levels of spatial interference. The results indicate that CGG KI mice showed difficulty performing a spatial novelty detection task when there were high levels of spatial interference, but were able to perform the novelty detection task when there was low spatial interference. These data suggest that CGG KI mice show reduced spatial and temporal resolution that are modulated by the dosage of the Fmr1 gene mutation, such that when behavioral tasks require mice to overcome high levels of either spatial or temporal interference, the CGG KI mice perform increasingly poorly as the CGG repeat length increases.",
keywords = "CGG KI mouse, Fragile X premutation, Pattern separation, Spatiotemporal processing, Temporal order",
author = "Borthwell, {Rachel M.} and Hunsaker, {Michael R.} and Rob Willemsen and Berman, {Robert F}",
year = "2012",
month = "7",
day = "15",
doi = "10.1016/j.bbr.2012.04.029",
language = "English (US)",
volume = "233",
pages = "29--34",
journal = "Behavioural Brain Research",
issn = "0166-4328",
publisher = "Elsevier",
number = "1",

}

TY - JOUR

T1 - Spatiotemporal processing deficits in female CGG KI mice modeling the fragile X premutation

AU - Borthwell, Rachel M.

AU - Hunsaker, Michael R.

AU - Willemsen, Rob

AU - Berman, Robert F

PY - 2012/7/15

Y1 - 2012/7/15

N2 - The fragile X premutation is a tandem CGG trinucleotide repeat expansion in the fragile X mental retardation 1 (FMR1) gene between 55 and 200 repeats in length. A CGG knock-in (CGG KI) mouse has been developed that models the neuropathology and cognitive deficits reported in fragile X premutation carriers. It has been suggested that carriers of the premutation demonstrate a spatiotemporal hypergranularity, or reduced resolution of spatial and temporal processing. A temporal ordering of spatial locations task was used to evaluate the ability of CGG KI mice to process temporal and spatial information with either high or low levels of spatial interference. The results indicate that CGG KI mice showed difficulty performing a spatial novelty detection task when there were high levels of spatial interference, but were able to perform the novelty detection task when there was low spatial interference. These data suggest that CGG KI mice show reduced spatial and temporal resolution that are modulated by the dosage of the Fmr1 gene mutation, such that when behavioral tasks require mice to overcome high levels of either spatial or temporal interference, the CGG KI mice perform increasingly poorly as the CGG repeat length increases.

AB - The fragile X premutation is a tandem CGG trinucleotide repeat expansion in the fragile X mental retardation 1 (FMR1) gene between 55 and 200 repeats in length. A CGG knock-in (CGG KI) mouse has been developed that models the neuropathology and cognitive deficits reported in fragile X premutation carriers. It has been suggested that carriers of the premutation demonstrate a spatiotemporal hypergranularity, or reduced resolution of spatial and temporal processing. A temporal ordering of spatial locations task was used to evaluate the ability of CGG KI mice to process temporal and spatial information with either high or low levels of spatial interference. The results indicate that CGG KI mice showed difficulty performing a spatial novelty detection task when there were high levels of spatial interference, but were able to perform the novelty detection task when there was low spatial interference. These data suggest that CGG KI mice show reduced spatial and temporal resolution that are modulated by the dosage of the Fmr1 gene mutation, such that when behavioral tasks require mice to overcome high levels of either spatial or temporal interference, the CGG KI mice perform increasingly poorly as the CGG repeat length increases.

KW - CGG KI mouse

KW - Fragile X premutation

KW - Pattern separation

KW - Spatiotemporal processing

KW - Temporal order

UR - http://www.scopus.com/inward/record.url?scp=84861549089&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84861549089&partnerID=8YFLogxK

U2 - 10.1016/j.bbr.2012.04.029

DO - 10.1016/j.bbr.2012.04.029

M3 - Article

C2 - 22561129

AN - SCOPUS:84861549089

VL - 233

SP - 29

EP - 34

JO - Behavioural Brain Research

JF - Behavioural Brain Research

SN - 0166-4328

IS - 1

ER -