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The Role of the Postsynaptic Density in the Pathology of the Fragile X 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 protein repertoire of excitatory synapses controls dendritic spine morphology, synaptic plasticity and higher brain functions. In brain neurons, the RNA-associated fragile X mental retardation protein (FMRP) binds in vivo to various transcripts encoding key postsynaptic components and may thereby substantially regulate the molecular composition of dendritic spines. In agreement with this notion functional loss of FMRP in patients affected by the fragile X syndrome (FXS) causes cognitive impairment. Here we address our current understanding of the functional role of individual postsynaptic proteins. We discuss how FMRP controls the abundance of select proteins at postsynaptic sites, which signaling pathways regulate the local activity of FMRP at synapses, and how altered levels of postsynaptic proteins may contribute to FXS pathology.

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Acknowledgments

Work in the authors’ laboratories is supported by grants from FRAXA, Deutsche Forschungsgemeinschaft, and Fritz-Thyssen-Stiftung.

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Authors and Affiliations

  1. Institute for Human Genetics, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany

    Stefan Kindler & Hans-Jürgen Kreienkamp

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  1. Stefan Kindler
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  2. Hans-Jürgen Kreienkamp
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Correspondence to Stefan Kindler .

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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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Kindler, S., Kreienkamp, HJ. (2012). The Role of the Postsynaptic Density in the Pathology of the Fragile X 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_5

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