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

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.