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First published online July 27, 2007

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Ellis-van Creveld (EvC) syndrome causes severe skeletal and craniofacial abnormalities, and Victor Ruiz-Perez, Judith Goodship and colleagues previously cloned two genes - EVC and EVC2 - that underlie this disorder. Now, on p. 2903, the researchers confirm that Evc ablation in mice results in an EvC-like phenotype and they use this mouse model to determine the molecular and developmental roles of Evc. They show that Evc is expressed in developing bones and in the orofacial region (the mouth and face). Within chondrocytes, the protein is localised to the primary cilium - an organelle that is central to hedgehog (Hh) signalling - which piqued the researchers' interest in Evc's relationship with Indian hedgehog (Ihh), a `master regulator' of bone development. Ihh itself is unaffected in Evc^-/- mice, but downstream genes, such as Ptch1 and Gli1, have reduced expression, and the researchers conclude that Evc is required for transducing the Ihh signal. Curiously, Evc^-/- mice have normal Gli3 processing. Next, the authors are going to use their model to find out whether other cell lineages rely on Evc for Hh signal transduction.

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Related articles in Development:

Evc is a positive mediator of Ihh-regulated bone growth that localises at the base of chondrocyte cilia

Victor L. Ruiz-Perez, Helen J. Blair, M. Elena Rodriguez-Andres, Maria Jose Blanco, Amy Wilson, Yu-Ning Liu, Colin Miles, Heiko Peters, and Judith A. Goodship
Development 2007 134: 2903-2912. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in Development Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?