| Mouse models of the fragile x premutation and the fragile x associated tremor/ataxia syndrome. | |
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MedLine Citation:
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PMID: 22009357 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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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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Authors:
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Michael R Hunsaker; Gloria Arque; Robert F Berman; Rob Willemsen; Renate K Hukema |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Results and problems in cell differentiation Volume: 54 ISSN: 0080-1844 ISO Abbreviation: Results Probl Cell Differ Publication Date: 2012 |
Date Detail:
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Created Date: 2011-10-19 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0173555 Medline TA: Results Probl Cell Differ Country: Germany |
Other Details:
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Languages: eng Pagination: 255-69 Citation Subset: IM |
Affiliation:
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Department of Neurological Surgery, University of California, Davis, CA, USA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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