PAR-Complex and Crumbs Function During Photoreceptor Morphogenesis and Retinal Degeneration.
PAR-Complex and Crumbs Function During Photoreceptor Morphogenesis and Retinal Degeneration.
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DOI：
10.3389/fncel.2018.00090
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发表时间：
2018
影响因子：
5.3
通讯作者：
Pichaud F
中科院分区：
文献类型：
--
作者：
Pichaud F
长期以来，苍蝇光感受器一直被用作研究感觉神经元形态发生和视网膜变性的模型。特别是，阐明这些细胞是如何构建的继续有助于我们进一步了解极化细胞形态发生、细胞内运输的机制以及人类视网膜病变的原因。在果蝇中，由CDC42-PAR6-aPKC-Bazooka组成的保守的PAR复合体和跨膜蛋白Crumbs(CRB)是光感受器形态发生过程中的关键角色。虽然PAR复合体在许多细胞类型中调节极性，但CRB在极性上的功能是相对特定于上皮细胞的。CDC42-PAR6-aPKC-Bazooka和CRB共同协调光感受器顶膜(AM)和贴壁带(ZA)的分化，从而允许这些细胞组装成神经上皮格子。除了在上皮极性中的作用外，CRB还被证明可以保护苍蝇的光感受器免受光诱导的退化，这一过程与视紫红质的表达和运输有关。值得注意的是，人类Crums1(CRB1)基因的突变会导致视网膜退化，使苍蝇光感受器成为一个强大的疾病模型系统。
The fly photoreceptor has long been used as a model to study sensory neuron morphogenesis and retinal degeneration. In particular, elucidating how these cells are built continues to help further our understanding of the mechanisms of polarized cell morphogenesis, intracellular trafficking and the causes of human retinal pathologies. The conserved PAR complex, which in flies consists of Cdc42-PAR6-aPKC-Bazooka, and the transmembrane protein Crumbs (Crb) are key players during photoreceptor morphogenesis. While the PAR complex regulates polarity in many cell types, Crb function in polarity is relatively specific to epithelial cells. Together Cdc42-PAR6-aPKC-Bazooka and Crb orchestrate the differentiation of the photoreceptor apical membrane (AM) and zonula adherens (ZA), thus allowing these cells to assemble into a neuro-epithelial lattice. In addition to its function in epithelial polarity, Crb has also been shown to protect fly photoreceptors from light-induced degeneration, a process linked to Rhodopsin expression and trafficking. Remarkably, mutations in the human Crumbs1 (CRB1) gene lead to retinal degeneration, making the fly photoreceptor a powerful disease model system.
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