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Mouse Models of the Fragile X Premutation and the Fragile X Associated Tremor/Ataxia Syndrome

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Book cover Modeling Fragile X Syndrome

Part of the book series: Results and Problems in Cell Differentiation ((RESULTS,volume 54))

Abstract

The use of mutant mouse models of neurodevelopmental and neurodegenerative disease is essential in order to understand the pathogenesis of many genetic diseases such as fragile X syndrome and fragile X-associated tremor/ataxia syndrome (FXTAS). The choice of which animal model is most suitable to mimic a particular disease depends on a range of factors, including anatomical, physiological, and pathological similarities; presence of orthologs of genes of interest; and conservation of basic cell biological and metabolic processes. In this chapter, we will discuss two mouse models of the fragile X premutation which have been generated to study the pathogenesis of FXTAS and the effects of potential therapeutic interventions. Behavioral, molecular, neuropathological, and endocrine features of the mouse models and their relation to human FXTAS are discussed.

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Acknowledgements

This work was made possible by a Roadmap Initiative grant (UL1 DE019583) from the NIDCR in support of the NeuroTherapeutics Research Institute (NTRI) consortium and Grant RL1 NS064211 from NINDS. This work was also supported by a grant (UL1 RR024146) from the National Center for Research Resources, a component of the National Institute of Health, and NIH Roadmap for Medical Research. This work was also supported by a research grant from the National Fragile X Foundation (RKH).

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Authors and Affiliations

  1. Department of Neurological Surgery, University of California, Davis, CA, USA

    Michael R. Hunsaker & Robert F. Berman

  2. NeuroTherapeutics Research Institute (NTRI), University of California, Davis, CA, USA

    Michael R. Hunsaker, Gloria Arque, Robert F. Berman & Rob Willemsen

  3. Department of Psychiatry and Behavioral Sciences, University of California, Davis, CA, USA

    Gloria Arque

  4. Department of Clinical Genetics, Erasmus MC, 2040, 3000 CA, Rotterdam, The Netherlands

    Rob Willemsen & Renate K. Hukema

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  1. Michael R. Hunsaker
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  2. Gloria Arque
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  3. Robert F. Berman
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  4. Rob Willemsen
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  5. Renate K. Hukema
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Correspondence to Rob Willemsen .

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  1. New York State Institute for Basic Resea, Forest Hill Road 1050, Staten Island, 10314, USA

    Robert B. Denman

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Hunsaker, M.R., Arque, G., Berman, R.F., Willemsen, R., Hukema, R.K. (2012). Mouse Models of the Fragile X Premutation and the Fragile X Associated Tremor/Ataxia Syndrome. In: Denman, R. (eds) Modeling Fragile X Syndrome. Results and Problems in Cell Differentiation, vol 54. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-21649-7_14

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