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The mouse model of the premutation (KI) was developed in the Laboratory of
Molecular and Cellular Biology, NIDDK under the direction of K. Usdin, PhD.
KI mice have the following characteristics:
- 120 CGG•CGG-repeats in the endogenous murine fmr1 gene
- Intranuclear neuronal inclusions
- Purkinje cell loss
- Elevated fmr1 mRNA
- Deficiency of FMRP in some brain regions (e.g., inferior olive)
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Our studies in adult male KI mice examine:
- behavior
- dendrite morphology
- regional rates of cerebral protein synthesis
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Preliminary results indicate that KI mice have the following behavioral deficits [1]:
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- Hyperactivity
- Impaired social interactions
- Reduced generalized anxiety
- Deficit in learning/memory
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