Deficiency of mDia, an Actin Nucleator, Disrupts Integrity of Neuroepithelium and Causes Periventricular Dysplasia
2011

mDia Deficiency Disrupts Neuroepithelial Integrity and Causes Brain Abnormalities

Sample size: 11 publication 10 minutes Evidence: high

Author Information

Author(s): Thumkeo Dean, Shinohara Ryota, Watanabe Keisuke, Takebayashi Hirohide, Toyoda Yosuke, Tohyama Kiyoshi, Ishizaki Toshimasa, Furuyashiki Tomoyuki, Narumiya Shuh

Primary Institution: Department of Pharmacology, Kyoto University Faculty of Medicine, Kyoto, Japan

Hypothesis

The study investigates the role of mDia, an actin nucleator, in maintaining the integrity of neuroepithelial cells during brain development.

Conclusion

Loss of mDia leads to impaired neuroepithelial cell polarity and the formation of periventricular dysplastic mass, which can cause hydrocephalus.

Supporting Evidence

  • mDia1 and mDia3 are expressed in the developing brain.
  • Mice deficient in both mDia1 and mDia3 develop periventricular dysplastic mass.
  • Electron microscopy revealed abnormal shrinkage and apical membrane bulging of neuroepithelial cells.
  • Perturbation of Rho causes loss of the apical actin belt and adherens junctions.
  • mDia deficiency leads to ectopic proliferation and differentiation of neural stem cells.

Takeaway

When a specific protein called mDia is missing, it causes problems in brain cell organization, leading to abnormal growths and fluid buildup in the brain.

Methodology

The researchers generated mice lacking mDia1 and mDia3 and analyzed the resulting brain structure and cell behavior through various histological techniques.

Limitations

The study primarily focuses on the effects of mDia deficiency in mice, which may not fully translate to human conditions.

Participant Demographics

Mice were used in the study, specifically mDia-deficient and control strains.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1371/journal.pone.0025465

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