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Curr Opin Genet Dev. 2011 Jun;21(3):333-9. doi: 10.1016/j.gde.2011.01.006. Epub 2011 Feb 1.

What disorders of cortical development tell us about the cortex: one plus one does not always make two.

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Division of Genetics, Manton Center for Orphan Disease Research and Howard Hughes Medical Institute, Children's Hospital Boston, Boston, MA 02115, United States.


The unique size and complexity of the human cerebral cortex are achieved via a long and precisely regulated developmental process controlling neurogenesis, neuronal migration and differentiation. Traditionally, disorders of cortical development have been classified on the basis of the most obvious defects in one of these developmental steps. However, the more we learn about the cellular biological roles of genes that are essential for cortical development, the more we realize that these functions map onto molecular processes, but not so cleanly onto anatomical processes. Essential genes might be involved in both proliferation and migration as well as differentiation, reflecting roles for underlying molecular mechanisms in different phases of development and causing a stunning variety of cortical defects.

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