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Control of Hedgehog Signalling by the Cilia-Regulated Proteasome

Institute for Animal Developmental and Molecular Biology, Heinrich-Heine University Düsseldorf, 40225 Düsseldorf, Germany
Author to whom correspondence should be addressed.
Academic Editors: Henk Roelink and Simon J. Conway
J. Dev. Biol. 2016, 4(3), 27;
Received: 31 May 2016 / Revised: 25 August 2016 / Accepted: 29 August 2016 / Published: 3 September 2016
(This article belongs to the Collection Hedgehog Signaling in Embryogenesis)
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The Hedgehog signalling pathway is evolutionarily highly conserved and essential for embryonic development of invertebrates and vertebrates. Consequently, impaired Hedgehog signalling results in very severe human diseases, ranging from holoprosencephaly to Pallister-Hall syndrome. Due to this great importance for human health, the focus of numerous research groups is placed on the investigation of the detailed mechanisms underlying Hedgehog signalling. Today, it is known that tiny cell protrusions, known as primary cilia, are necessary to mediate Hedgehog signalling in vertebrates. Although the Hedgehog pathway is one of the best studied signalling pathways, many questions remain. One of these questions is: How do primary cilia control Hedgehog signalling in vertebrates? Recently, it was shown that primary cilia regulate a special kind of proteasome which is essential for proper Hedgehog signalling. This review article will cover this novel cilia-proteasome association in embryonic Hedgehog signalling and discuss the possibilities provided by future investigations on this topic. View Full-Text
Keywords: ciliary proteasome; proteolytic processing; GLI2; GLI3; cilia; signalling; RPGRIP1L; mouse; Drosophila; SHH ciliary proteasome; proteolytic processing; GLI2; GLI3; cilia; signalling; RPGRIP1L; mouse; Drosophila; SHH

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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

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Gerhardt, C.; Wiegering, A.; Leu, T.; Rüther, U. Control of Hedgehog Signalling by the Cilia-Regulated Proteasome. J. Dev. Biol. 2016, 4, 27.

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