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Evc regulates a symmetrical response to Shh signaling in molar development

Lookup NU author(s): Dr Mitsushiro Nakatomi, Dr Helen Blair, Katie MacArthur, Dr Victor Ruiz-Perez, Professor Judith Goodship, Dr Heiko Peters


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Tooth morphogenesis involves patterning through the activity of epithelial signaling centers that, among other molecules, secrete Sonic hedgehog (Shh). While it is known that Shh responding cells need intact primary cilia for signal transduction, the roles of individual cilia components for tooth morphogenesis are poorly understood. The clinical features of individuals with Ellis-van Creveld syndrome include various dental anomalies, and we show here that absence of the cilial protein Evc in mice causes various hypo- and hyperplasia defects during molar development. During first molar development, the response to Shh signaling is progressively lost in Evc-deficient embryos and, unexpectedly, the response consistently disappears in a buccal to lingual direction. The important role of Evc for establishing the buccal-lingual axis of the developing first molar is also supported by a displaced activity of the Wnt pathway in Evc mutants. The observed growth abnormalities eventually manifest in first molar microdontia, disruption of molar segmentation and symmetry, root fusions, and delayed differentiation. Analysis of our data indicates that both spatially and temporally disrupted activities of the Shh pathway are the primary cause for the variable dental anomalies seen in patients with Ellis-van Creveld syndrome or Weyers acrodental dysostosis.

Publication metadata

Author(s): Nakatomi M, Hovorakova M, Gritli-Linde A, Blair HJ, Macarthur K, Peterka M, Lesot H, Peterkova R, Ruiz-Perez VL, Goodship JA, Peters H

Publication type: Article

Publication status: Published

Journal: Journal of Dental Research

Year: 2013

Volume: 92

Issue: 3

Pages: 222-228

Print publication date: 11/01/2013

ISSN (print): 0022-0345

ISSN (electronic): 1544-0591

Publisher: Sage Publications, Inc.


DOI: 10.1177/0022034512471826


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Funder referenceFunder name
JSPS, Japan
CZ:GACR:GA304/09/1579Grant Agency of the Czech Republic
CZ:GACR:GAP305/12/1766Grant Agency of the Czech Republic
G0400679MRC, UK