Format

Send to

Choose Destination
See comment in PubMed Commons below
Behav Neurosci. 2009 Dec;123(6):1315-24. doi: 10.1037/a0017616.

Progressive spatial processing deficits in a mouse model of the fragile X premutation.

Author information

1
Program in Neuroscience, University of California, Davis, Davis, CA 95616, USA.

Abstract

Fragile X associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder that is the result of a CGG trinucleotide repeat expansion in the range of 55-200 in the 5' UTR of the FMR1 gene. To better understand the progression of this disorder, a knock-in (CGG KI) mouse was developed by substituting the mouse CGG8 trinucleotide repeat with an expanded CGG98 repeat from human origin. It has been shown that this mouse shows deficits on the water maze at 52 weeks of age. In the present study, this CGG KI mouse model of FXTAS was tested on behavioral tasks that emphasize spatial information processing. The results demonstrate that at 12 and 24 weeks of age, CGG KI mice were unable to detect a change in the distance between two objects (metric task), but showed intact detection of a transposition of the objects (topological task). At 48 weeks of age, CGG KI mice were unable to detect either change in object location. These data indicate that hippocampal-dependent impairments in spatial processing may occur prior to parietal cortex-dependent impairments in FXTAS.

PMID:
20001115
PMCID:
PMC3410547
DOI:
10.1037/a0017616
[Indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for American Psychological Association Icon for PubMed Central
    Loading ...
    Support Center