| Dendritic spine structural anomalies in fragile-X mental retardation syndrome. | |
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MedLine Citation:
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PMID: 11007554 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Fragile-X syndrome is the most common single-gene inherited form of mental retardation. Morphological studies suggest a possible failure of the synapse maturation process. Cerebral cortical spine morphology in fragile-X syndrome and in a knockout mouse model of it appears immature, with long, thin spines much more common than the stubby and mushroom-shaped spines more characteristic of normal development. In human fragile-X syndrome there is also a higher density of spines along dendrites, suggesting a possible failure of synapse elimination. While variously misshapen spines are characteristic of a number of mental retardation syndromes, the overabundance of spines seen in fragile-X syndrome is unusual. Taken with evidence of neurotransmitter activation of the synthesis of the fragile-X protein (FMRP) at synapses in vitro and evidence for behaviorally induced FMRP expression in vivo, and with evidence compatible with a role for FMRP in regulating the synthesis of other proteins, it is possible that FMRP serves as an 'immediate early protein' at the synapse that orchestrates aspects of synaptic development and plasticity. |
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Authors:
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S A Irwin; R Galvez; W T Greenough |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.; Review |
Journal Detail:
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Title: Cerebral cortex (New York, N.Y. : 1991) Volume: 10 ISSN: 1047-3211 ISO Abbreviation: Cereb. Cortex Publication Date: 2000 Oct |
Date Detail:
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Created Date: 2000-10-16 Completed Date: 2000-10-25 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 9110718 Medline TA: Cereb Cortex Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1038-44 Citation Subset: IM |
Affiliation:
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Neuroscience and Medical Scholars Programs, Departments of Psychology, Psychiatry, and Cell and Structural Biology, and the Beckman Institute, University of Illinois, Urbana, IL 61801, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Brain
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pathology Dendrites / ultrastructure* Fragile X Mental Retardation Protein Fragile X Syndrome / complications* Humans Mental Retardation / genetics*, pathology* Nerve Tissue Proteins / physiology RNA-Binding Proteins* Synapses / physiology |
| Grant Support | |
ID/Acronym/Agency:
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HD 37175/HD/NICHD NIH HHS; MH 11272/MH/NIMH NIH HHS; MH 35321/MH/NIMH NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/FMR1 protein, human; 0/Nerve Tissue Proteins; 0/RNA-Binding Proteins; 139135-51-6/Fragile X Mental Retardation Protein |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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