【摘 要】
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Planar cell polarity(PCP)signaling plays a critical role in tissue morphogenesis.Previously,we have shown that Prickle 1,a key PCP component,is essential for oriented cell arrangement.Loss-of-function
【机 构】
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State Key Laboratory of Ophthalmology,Zhongshan Ophthalmic Center,Sun Yat-sen University
【出 处】
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第九届海内外华人神经科学家研讨会(The 9th Symposium for Chinese Neuroscientis
论文部分内容阅读
Planar cell polarity(PCP)signaling plays a critical role in tissue morphogenesis.Previously,we have shown that Prickle 1,a key PCP component,is essential for oriented cell arrangement.Loss-of-function of Prickle 1 phenocopies human Robinow Syndrome and disrupts non-canonical Wnt5a signaling.In this study,we examined role of Prickle in ocular development.Using combined Prickle 1 mutant alleles,we showed a significant decrease of a-and b-wave amplitudes in ERG recordings under both dark-and light-adapted conditions.Lens suture pattern and epithelial cell morphology are altered giving rise to congenital cataract.Photoreceptor outer segments appear disorganized.Staining for RGC markers with anti-Brain3a,anti-NeuN and anti-Neurofilament is attenuated.Retinal vasculature exhibits increased tortuosity and branching defects.Thus,reduced PK1 expression seems to affect all layers of the neural retina,the lens and extraocular tissues.The mechanisms underlying these defects are under investigation.
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