2011
DOI: 10.1155/2011/589414
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Planar Cell Polarity Signaling Pathway in Congenital Heart Diseases

Abstract: Congenital heart disease (CHD) is a common cardiac disorder in humans. Despite many advances in the understanding of CHD and the identification of many associated genes, the fundamental etiology for the majority of cases remains unclear. The planar cell polarity (PCP) signaling pathway, responsible for tissue polarity in Drosophila and gastrulation movements and cardiogenesis in vertebrates, has been shown to play multiple roles during cardiac differentiation and development. The disrupted function of PCP sign… Show more

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Cited by 21 publications
(16 citation statements)
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References 99 publications
(154 reference statements)
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“…PCP, to promote cell migration and convergent extension movements. 2,189,192,193,196 The link between Wnt signaling and the primary cilium is highly controversial. 32,35,197 However, many Wnt signaling components, such as Fz3, Dvl, and β-catenin, localize to the ciliary/centrosomal axis, and dysregulated Wnt signaling has been reported in cells and tissues with disrupted ciliogenesis or basal body integrity.…”
Section: Cardiac Primary Cilia and Other Signaling Pathwaysmentioning
confidence: 99%
See 3 more Smart Citations
“…PCP, to promote cell migration and convergent extension movements. 2,189,192,193,196 The link between Wnt signaling and the primary cilium is highly controversial. 32,35,197 However, many Wnt signaling components, such as Fz3, Dvl, and β-catenin, localize to the ciliary/centrosomal axis, and dysregulated Wnt signaling has been reported in cells and tissues with disrupted ciliogenesis or basal body integrity.…”
Section: Cardiac Primary Cilia and Other Signaling Pathwaysmentioning
confidence: 99%
“…2,189 Mutations in Vangl2 disrupt migration of cells into the OFT and this is associated with impaired OFT myocardialization as well as ventricular and OFT septal defects. 189 The localization of Vangl2 during heart development is dependent on Scribble, another PCP component, 204 and in agreement with this, Scribble phenotypes are reminiscent of Vangl2 mutants.…”
Section: Cardiac Primary Cilia and Other Signaling Pathwaysmentioning
confidence: 99%
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“…Disturbances in PCP signaling produce developmental anomalies in neural tube closure, left-right asymmetry, and hair cell orientation in the inner ear (14)(15)(16)(17)(18)(19)(20). In humans, mutations or polymorphisms of PCP components are associated with an array of developmental defects including spina bifida, cardiac outflow malformations, and cystic renal disease (21)(22)(23)(24).…”
Section: Significancementioning
confidence: 99%