Progressive Spatial Processing Deficits in a Mouse Model of the Fragile X Premutation
| Autor: | Rob Willemsen, H. Jürgen Wenzel, Michael R. Hunsaker, Robert F. Berman |
|---|---|
| Přispěvatelé: | Clinical Genetics |
| Rok vydání: | 2009 |
| Předmět: |
Male
congenital hereditary and neonatal diseases and abnormalities Ataxia Time Factors Genotype Spatial ability Intranuclear Inclusion Bodies Spatial Behavior Mice Transgenic Water maze Article Behavioral Neuroscience Fragile X Mental Retardation Protein Mice Parietal Lobe medicine Animals Analysis of Variance Behavior Animal Dentate gyrus Parietal lobe Age Factors Recognition Psychology Spatial cognition medicine.disease Immunohistochemistry nervous system diseases Fragile X syndrome Space Perception Dentate Gyrus Exploratory Behavior medicine.symptom Trinucleotide repeat expansion Psychology Trinucleotide Repeat Expansion Neuroscience |
| Zdroj: | Behavioral Neuroscience, 123(6), 1315-1324. American Psychological Association Inc. |
| ISSN: | 1939-0084 0735-7044 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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