The role of the postsynaptic density in the pathology of the fragile X syndrome.
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The role of the postsynaptic density in the pathology of the fragile X syndrome. / Kindler, Stefan; Kreienkamp, Hans-Jürgen.
In: Results Probl Cell Differ, Vol. 54, 2012, p. 61-80.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - The role of the postsynaptic density in the pathology of the fragile X syndrome.
AU - Kindler, Stefan
AU - Kreienkamp, Hans-Jürgen
PY - 2012
Y1 - 2012
N2 - 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.
AB - 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.
KW - Animals
KW - Humans
KW - Gene Expression Regulation
KW - RNA, Messenger/metabolism
KW - Membrane Proteins/genetics/metabolism
KW - Fragile X Mental Retardation Protein/genetics/metabolism
KW - Fragile X Syndrome/pathology/physiopathology
KW - Post-Synaptic Density/physiology/ultrastructure
KW - Animals
KW - Humans
KW - Gene Expression Regulation
KW - RNA, Messenger/metabolism
KW - Membrane Proteins/genetics/metabolism
KW - Fragile X Mental Retardation Protein/genetics/metabolism
KW - Fragile X Syndrome/pathology/physiopathology
KW - Post-Synaptic Density/physiology/ultrastructure
M3 - SCORING: Journal article
VL - 54
SP - 61
EP - 80
ER -