Genes that Control the Size of the Cerebral Cortex

  1. Gregory Bock Organizer and
  2. Jamie Goode
  1. Teresa H. Chae1,2 and
  2. Christopher A. Walsh1,2

Published Online: 1 FEB 2008

DOI: 10.1002/9780470994030.ch6

Cortical Development: Genes and Genetic Abnormalities: Novartis Foundation Symposium 288

Cortical Development: Genes and Genetic Abnormalities: Novartis Foundation Symposium 288

How to Cite

Chae, T. H. and Walsh, C. A. (2008) Genes that Control the Size of the Cerebral Cortex, in Cortical Development: Genes and Genetic Abnormalities: Novartis Foundation Symposium 288 (eds G. Bock and J. Goode), John Wiley & Sons, Ltd, Chichester, UK. doi: 10.1002/9780470994030.ch6

Author Information

  1. 1

    Howard Hughes Medical Institute, Beth Israel Deaconess Medical Center; Division of Genetics, Children's Hospital, Boston, MA 02115, USA

  2. 2

    Program in Neuroscience, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA

Publication History

  1. Published Online: 1 FEB 2008
  2. Published Print: 11 JAN 2008

Book Series:

  1. Novartis Foundation Symposia

Book Series Editors:

  1. Novartis Foundation

ISBN Information

Print ISBN: 9780470060926

Online ISBN: 9780470994030

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Keywords:

  • neural progenitor proliferation and differentiation;
  • symmetric and asymmetric cell divisions;
  • cell fate determinants;
  • genes controlling cortical size;
  • ganglion mother cell (GMC);
  • vertebrate nervous system;
  • mitotic spindle orientation;
  • apical–lateral cell surface;
  • SNARE-mediated vesicle trafficking and intracellular membrane fusion;
  • abnormal spindle-like microcephaly (ASPM)

Summary

Study of the mechanisms that control growth of the cerebral cortex has largely followed by analogy from work in invertebrate systems such as fly and worm. However, the identification of several genes that cause human microcephaly has provided new avenues of investigation into the mechanisms that control cell identity during cerebral cortical development. In vivo studies suggest that many forms of microcephaly result from defects in the control of cell fate: precocious formation of neurons during early developmental stages produces deficiencies in progenitor cells at later stages of neurogenesis, resulting in an overall small cerebral cortex. Also, some of the genes that are mutated in human microcephaly seem to have been targets in the evolution of humans from distant primate ancestors.