Document Detail

Localization of the fragile X mental retardation 2 (FMR2) protein in mammalian brain.
MedLine Citation:
PMID:  10651894     Owner:  NLM     Status:  MEDLINE    
The transcriptional silencing of the FMR2 gene has been implicated in FRAXE mental retardation. FRAXE individuals have been shown to exhibit learning deficits, including speech delay, reading and writing problems. FMR2 encodes a large protein of 1311 amino acids and is a member of a gene family encoding proline-serine-rich proteins that have properties of nuclear transcription factors. To characterize the expression of the fragile X mental retardation 2 (FMR2) protein, polyclonal antibodies were raised against two regions of the human FMR2 protein and used in immunofluorescence experiments on mouse brain cryosections. Our results demonstrate for the first time that the FMR2 protein is localized in neurons of the neocortex, Purkinje cells of the cerebellum and the granule cell layer of the hippocampus. FMR2 staining is shown to colocalize with the nuclear stain 4,6-diamidino-2-phenylindole (DAPI) confirming that FMR2 is a nuclear protein. The localization of FMR2 protein to the mammalian hippocampus and other brain structures involved with cognitive function is consistent with the learning deficits seen in FRAXE individuals.
W J Miller; J A Skinner; G S Foss; K E Davies
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The European journal of neuroscience     Volume:  12     ISSN:  0953-816X     ISO Abbreviation:  Eur. J. Neurosci.     Publication Date:  2000 Jan 
Date Detail:
Created Date:  2000-03-16     Completed Date:  2000-03-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8918110     Medline TA:  Eur J Neurosci     Country:  FRANCE    
Other Details:
Languages:  eng     Pagination:  381-4     Citation Subset:  IM    
Department of Human Anatomy, University of Oxford, South Parks Road, Oxford OX1 3QX, UK.
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MeSH Terms
Brain / cytology*
Cloning, Molecular
Escherichia coli
Fragile X Syndrome / genetics
Mice, Inbred C57BL
Nuclear Proteins*
Proteins / analysis*,  genetics
Recombinant Proteins / analysis
Reg. No./Substance:
0/AFF2 protein, human; 0/Aff2 protein, mouse; 0/Antibodies; 0/Nuclear Proteins; 0/Proteins; 0/Recombinant Proteins; 0/Trans-Activators

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