You are here

Cdk5rap2 exposes the centrosomal root of microcephaly syndromes.

Title: Cdk5rap2 exposes the centrosomal root of microcephaly syndromes.
17 views
7 downloads
Name(s): Megraw, Timothy L, author
Sharkey, James T, author
Nowakowski, Richard S, author
Type of Resource: text
Genre: Journal Article
Text
Date Issued: 2011-08-01
Physical Form: computer
online resource
Extent: 1 online resource
Language(s): English
Abstract/Description: 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.
Identifier: FSU_pmch_21632253 (IID), 10.1016/j.tcb.2011.04.007 (DOI), PMC3371655 (PMCID), 21632253 (RID), 21632253 (EID), S0962-8924(11)00083-3 (PII)
Grant Number: R01 GM068756-06A1, R01GM068756, R01 GM068756-06A1S1, R01 GM068756-08, GM068756, R01 GM068756, R01 GM068756-07W1, R01 GM068756-07
Publication Note: This NIH-funded author manuscript originally appeared in PubMed Central at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3371655.
Subject(s): Animals
Centrosome/metabolism
Humans
Intracellular Signaling Peptides and Proteins/genetics
Intracellular Signaling Peptides and Proteins/metabolism
Mice
Microcephaly/genetics
Microcephaly/metabolism
Microcephaly/pathology
Microtubule-Associated Proteins/genetics
Microtubule-Associated Proteins/metabolism
Nerve Tissue Proteins/genetics
Nerve Tissue Proteins/metabolism
Spindle Apparatus/pathology
Persistent Link to This Record: http://purl.flvc.org/fsu/fd/FSU_pmch_21632253
Owner Institution: FSU
Is Part Of: Trends in cell biology.
1879-3088
Issue: iss. 8, vol. 21

Choose the citation style.
Megraw, T. L., Sharkey, J. T., & Nowakowski, R. S. (2011). Cdk5rap2 exposes the centrosomal root of microcephaly syndromes. Trends In Cell Biology. Retrieved from http://purl.flvc.org/fsu/fd/FSU_pmch_21632253