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Publication : Somatosensory cortical barrel dendritic abnormalities in a mouse model of the fragile X mental retardation syndrome.

First Author  Galvez R Year  2003
Journal  Brain Res Volume  971
Issue  1 Pages  83-9
PubMed ID  12691840 Mgi Jnum  J:83158
Mgi Id  MGI:2657086 Doi  10.1016/s0006-8993(03)02363-1
Citation  Galvez R, et al. (2003) Somatosensory cortical barrel dendritic abnormalities in a mouse model of the fragile X mental retardation syndrome. Brain Res 971(1):83-9
abstractText  The Fragile X mental retardation syndrome is the largest source of inherited mental retardation. The syndrome usually results from the transcriptional silencing of the fragile X mental retardation gene (FMR1). To date the most prominent reported neuronal abnormalities for the fragile X mental retardation syndrome include a higher density of long thin spines similar to those found in sensory deprived and developing tissue, suggesting a possible deficit in pruning of immature spines. Dendrites on spiny stellate cells in the inner 1/3 of the barrel wall in layer IV of the rodent somatosensory cortex have been shown to exhibit developmental pruning similar to that affecting spines. To determine if FMRP plays a role in dendritic development, these neurons were examined in two strains of adult FMRP knockout (FraX) mice. FraX mice in both strains exhibited a greater amount of septa-oriented dendritic material, a morphology consistent with pre-pruning status early in development. This observation suggests that FMRP could be necessary for normal developmentally regulated dendritic pruning.
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