Dysregulation of Rho GTPases in the αPix/Arhgef6 mouse model of X-linked intellectual disability is paralleled by impaired structural and synaptic plasticity and cognitive deficits.

@article{Ramakers2012DysregulationOR,
  title={Dysregulation of Rho GTPases in the αPix/Arhgef6 mouse model of X-linked intellectual disability is paralleled by impaired structural and synaptic plasticity and cognitive deficits.},
  author={Ger J. A. Ramakers and David P Wolfer and Georg Rosenberger and Kerstin Kuchenbecker and H J Kreienkamp and Janine Prange-Kiel and Gabriele M Rune and K. -D. Richter and Kristina Langnaese and Sophie Masneuf and Michael R. B{\"o}sl and Klaus-Dieter Fischer and Harm J. Krugers and Hans-Peter Lipp and Elly J M van Galen and Kerstin Kutsche},
  journal={Human molecular genetics},
  year={2012},
  volume={21 2},
  pages={268-86}
}
Mutations in the ARHGEF6 gene, encoding the guanine nucleotide exchange factor αPIX/Cool-2 for the Rho GTPases Rac1 and Cdc42, cause X-linked intellectual disability (ID) in humans. We show here that αPix/Arhgef6 is primarily expressed in neuropil regions of the hippocampus. To study the role of αPix/Arhgef6 in neuronal development and plasticity and gain insight into the pathogenic mechanisms underlying ID, we generated αPix/Arhgef6-deficient mice. Gross brain structure in these mice appeared… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 39 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 92 references

Similar Papers

Loading similar papers…