title: Hedgehog trafficking, cilia and brain functions.
abstract: The primary cilium has recently emerged as an important center for transduction of the Sonic Hedgehog (Shh) signal. Genetic studies have shown that Shh signaling at the level of primary cilia is essential for patterning the ventral neural tube and regulating adult stem cells. Some defects observed in human diseases and resulting from mutations affecting the organization of the primary cilium have been attributed to defective Shh signaling. The recent development of Shh pathway inhibitors for treating tumors linked to perturbations of Shh signaling has fostered studies to understand their mechanism of action in Shh receptor complex trafficking at the primary cilium.
authors.affiliation: CNRS, UPR-3294, Laboratoire de Neurobiologie et Développement, Institut de Neurobiologie Alfred Fessard IFR2118, Signal Transduction and Developmental Neuropharmacology Team, 1 avenue de la Terrasse, F-91198 Gif-sur-Yvette, France. ruat@inaf.cnrs-gif.fr
authors.forename: Martial
authors.initials: M
authors.lastname: Ruat
authors.forename: Hermine
authors.initials: H
authors.lastname: Roudaut
authors.forename: Julien
authors.initials: J
authors.lastname: Ferent
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Traiffort
mesh_terms: D000818:Animals; D001921:Brain / Q000254:growth & development / Q000502:physiology*; D002923:Cilia / Q000502:physiology*; D002925:Ciliary Motility Disorders / Q000235:genetics / Q000378:metabolism; D053823:Hedgehog Proteins / Q000378:metabolism*; D006801:Humans; D021381:Protein Transport; D015398:Signal Transduction
chemical_list: D053823:Hedgehog Proteins