The role of primary cilia in neuronal function

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dc.contributor.authorLee, Jeong Hoko
dc.contributor.authorGleeson, Joseph G.ko
dc.date.accessioned2013-03-13T03:04:29Z-
dc.date.available2013-03-13T03:04:29Z-
dc.date.created2012-10-14-
dc.date.created2012-10-14-
dc.date.issued2010-05-
dc.identifier.citationNEUROBIOLOGY OF DISEASE, v.38, pp.167 - 172-
dc.identifier.issn0969-9961-
dc.identifier.urihttp://hdl.handle.net/10203/104322-
dc.description.abstractThe "ciliopathies" are a newly defined group of disorders characterized by defects in the structure or function of the cellular primary cilium. Patients with these disorders display variably expressive fibrocystic renal disease, retinal blindness, polydactyly, obesity, and brain dysgenesis as well as neurocognitive impairments. Joubert syndrome is a ciliopathy defined by cerebellar vermis hypoplasia, oculomotor apraxia, intermittent hyperventilation, and mental retardation. Recent evidence suggests important roles for the primary cilium in mediating a host of extracellular signaling events such as morphogen, mitogen, homeostatic and polarity signals. Based upon the clinical features of ciliopathies and cilia mediated signaling pathways, the data support a role for the primary cilium in modulating neurogenesis, cell polarity, axonal guidance and possibly adult neuronal function. (C) 2009 Elsevier Inc. All rights reserved.-
dc.languageEnglish-
dc.publisherACADEMIC PRESS INC ELSEVIER SCIENCE-
dc.subjectBARDET-BIEDL-SYNDROME-
dc.subjectINTRAFLAGELLAR TRANSPORT PROTEINS-
dc.subjectPOLYCYSTIC KIDNEY-DISEASE-
dc.subjectJOUBERT-SYNDROME-
dc.subjectSONIC HEDGEHOG-
dc.subjectCENTROSOMAL PROTEIN-
dc.subjectCOUPLED-RECEPTORS-
dc.subjectPROGENITOR POOL-
dc.subjectCELL POLARITY-
dc.subjectC-ELEGANS-
dc.titleThe role of primary cilia in neuronal function-
dc.typeArticle-
dc.identifier.wosid000277253900004-
dc.identifier.scopusid2-s2.0-77951208687-
dc.type.rimsART-
dc.citation.volume38-
dc.citation.beginningpage167-
dc.citation.endingpage172-
dc.citation.publicationnameNEUROBIOLOGY OF DISEASE-
dc.identifier.doi10.1016/j.nbd.2009.12.022-
dc.contributor.localauthorLee, Jeong Ho-
dc.contributor.nonIdAuthorGleeson, Joseph G.-
dc.type.journalArticleReview-
dc.subject.keywordAuthorCiliopathy-
dc.subject.keywordAuthorJoubert syndrome-
dc.subject.keywordAuthorNeuronal function-
dc.subject.keywordAuthorPrimary cilia-
dc.subject.keywordPlusBARDET-BIEDL-SYNDROME-
dc.subject.keywordPlusINTRAFLAGELLAR TRANSPORT PROTEINS-
dc.subject.keywordPlusPOLYCYSTIC KIDNEY-DISEASE-
dc.subject.keywordPlusJOUBERT-SYNDROME-
dc.subject.keywordPlusSONIC HEDGEHOG-
dc.subject.keywordPlusCENTROSOMAL PROTEIN-
dc.subject.keywordPlusCOUPLED-RECEPTORS-
dc.subject.keywordPlusPROGENITOR POOL-
dc.subject.keywordPlusCELL POLARITY-
dc.subject.keywordPlusC-ELEGANS-
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