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Neural Stem Cells

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Modeling Fragile X Syndrome

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

Abstract

Neural stem/progenitor cell (NPC) cultures are a tool to study the differentiation of neuronal cells and can be used to model disease conditions in studies investigating the pathological mechanisms affecting the development and cellular plasticity of the central nervous system. There is evidence that abnormalities of NPCs and their differentiation contribute to the pathophysiology of fragile X syndrome. The results obtained with NPC cultures derived from human and mouse brain tissue with the fragile X mutation are in line with the abnormalities of Fmr1-knockout mouse brain in vivo indicating that NPC cultures can be useful as a model for fragile X syndrome.

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Abbreviations

aNPCs:

Adult neural precursor cells

bFGF:

Basic fibroblastic growth factor

CNS:

Central nervous system

EGF:

Epidermal growth factor

ES:

Embryonic cells

FXS:

Fragile X syndrome

IPC:

Intermediate progenitor cell

iPS cells:

Induced pluripotent stem cells

NPC:

Neural precursor cell

RG:

Radial glia

SVZ:

Subventricular zone

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Acknowledgment

The author thanks Dr. William Greenough for support to FXS stem cell studies.

Author information

Authors and Affiliations

  1. Institute of Biomedicine/Physiology, University of Helsinki, 63, Helsinki, Finland

    Maija Castrén

  2. Rinnekoti Foundation, Rinnekodintie 10, 02980, Espoo, Finland

    Maija Castrén

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  1. Maija Castrén
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Correspondence to Maija Castrén .

Editor information

Editors and Affiliations

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

    Robert B. Denman

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© 2012 Springer-Verlag Berlin Heidelberg

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Castrén, M. (2012). Neural Stem Cells. 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_3

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