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Philos Trans R Soc Lond B Biol Sci. 2014 Sep 5;369(1650). pii: 20130468. doi: 10.1098/rstb.2013.0468.

Small organelle, big responsibility: the role of centrosomes in development and disease.

Author information

1
Cancer Research UK Cambridge Institute, Li Ka Shing Centre, Robinson Way, Cambridge CB2 0RE, UK.
2
Cancer Research UK Cambridge Institute, Li Ka Shing Centre, Robinson Way, Cambridge CB2 0RE, UK fanni.gergely@cruk.cam.ac.uk.

Abstract

The centrosome, a key microtubule organizing centre, is composed of centrioles, embedded in a protein-rich matrix. Centrosomes control the internal spatial organization of somatic cells, and as such contribute to cell division, cell polarity and migration. Upon exiting the cell cycle, most cell types in the human body convert their centrioles into basal bodies, which drive the assembly of primary cilia, involved in sensing and signal transduction at the cell surface. Centrosomal genes are targeted by mutations in numerous human developmental disorders, ranging from diseases exclusively affecting brain development, through global growth failure syndromes to diverse pathologies associated with ciliary malfunction. Despite our much-improved understanding of centrosome function in cellular processes, we know remarkably little of its role in the organismal context, especially in mammals. In this review, we examine how centrosome dysfunction impacts on complex physiological processes and speculate on the challenges we face when applying knowledge generated from in vitro and in vivo model systems to human development.

KEYWORDS:

centriole; centrosome; cilia; ciliopathy; dwarfism; microcephaly

PMID:
25047622
PMCID:
PMC4113112
DOI:
10.1098/rstb.2013.0468
[Indexed for MEDLINE]
Free PMC Article

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