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Microcephaly Proteins Wdr62 and Aspm Define a Mother Centriole Complex Regulating Centriole Biogenesis, Apical

Divya Jayaraman1, Andrew Kodani2, Dilenny M Gonzalez3

  • 1Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA 02115, USA; Manton Center for Orphan Disease Research, Boston Children's Hospital, Boston, MA 02115, USA; Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA; Program in Neuroscience, Harvard Medical School, Boston, MA 02115, USA; Harvard-MIT MD-PhD Program, Harvard Medical School, Boston, MA 02115, USA.

Neuron
|December 16, 2016
PubMed

Insights

Abnormal spindle-like (Aspm) and WD repeat-containing protein 62 (Wdr62) proteins control human brain size by regulating centriole duplication and progenitor cell division. Defects in these proteins lead to microcephaly, demonstrating their critical role in brain development.

Area of Science:

  • Developmental biology
  • Cell biology
  • Genetics

Background:

  • Mutations in centrosomal protein genes are linked to reduced human brain size (microcephaly).
  • The specific roles of Aspm and Wdr62 in brain size regulation and progenitor cell behavior are not fully understood.

Purpose of the Study:

  • To investigate the genetic and physical interactions between Aspm and Wdr62 in controlling brain size.
  • To elucidate the molecular mechanisms by which Aspm and Wdr62 influence centriole duplication and progenitor cell differentiation.

Main Methods:

  • Genetic analysis in mice lacking Wdr62, Aspm, or both.
  • Immunolocalization studies to determine protein localization at the mother centriole.
  • Analysis of centriole duplication, progenitor cell delamination, and apical epithelial structure.

Main Results:

  • Aspm and Wdr62 interact genetically, and their absence causes gene dose-dependent centriole duplication defects and microcephaly in mice.
  • Wdr62 is required for Aspm localization to the mother centriole; both are essential for the localization of CENPJ/CPAP/Sas-4.
  • Aspm and Wdr62 are crucial for apical complex localization and apical epithelial structure, explaining premature progenitor delamination and differentiation.

Conclusions:

  • Aspm and Wdr62 form a critical complex at the mother centriole, regulating centriole duplication and progenitor cell fate during brain development.
  • This interaction highlights a link between centrioles, apical proteins, and cell fate, providing a mechanism for brain size regulation.
Keywords:
AspmWdr62apical complexmaternal centriole

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