Cdk5rap2 exposes the centrosomal root of microcephaly syndromes.

Timothy L Megraw, James T Sharkey, Richard S Nowakowski

Department of Biomedical Sciences, Florida State University, College of Medicine, Tallahassee, USA. timothy.megraw@med.fsu.edu

Trends in cell biology 2011 Aug

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Autosomal recessive primary microcephaly (MCPH) is characterized by small brain size as a result of deficient neuron production in the developing cerebral cortex. Although MCPH is a rare disease, the questions surrounding its etiology strike at the core of stem cell biology. The seven genes implicated in MCPH all encode centrosomal proteins and disruption of the MCPH gene Cdk5rap2 in mice revealed its role in neural progenitor proliferation and in maintaining normal centriole replication control. We discuss here the impact that centrosome regulation has upon neural progenitors in the developing brain. We integrate the impact of centriole replication defects with the functions of Cdk5rap2 and other MCPH proteins, propose mechanisms for progenitor loss in MCPH, and discuss links to two other microcephaly syndromes. Copyright © 2011 Elsevier Ltd. All rights reserved.

Citation

Timothy L Megraw, James T Sharkey, Richard S Nowakowski. Cdk5rap2 exposes the centrosomal root of microcephaly syndromes. Trends in cell biology. 2011 Aug;21(8):470-80