Lunardi Andrea, Cremisi Federico, Dente Luciana
Dipartimento di Fisiologia e Biochimica, Laboratori di Biologia Cellulare e dello Sviluppo, Università di Pisa, via G. Carducci 13, Ghezzano, Pisa 56010, Italy.
Dev Biol. 2006 Feb 15;290(2):411-20. doi: 10.1016/j.ydbio.2005.11.044. Epub 2006 Jan 9.
Dystroglycan (DG) is a transmembrane receptor linking the extracellular matrix to the internal cytoskeleton. Its structural function has been mainly characterized in muscle fibers, but DG plays signaling and developmental roles also in different tissues and cell types. We have investigated the effects of dystroglycan depletion during eye development of Xenopus laevis. We have injected a specific morpholino (Mo) antisense oligonucleotide in the animal pole of one dorsal blastomere of embryos at four cells stage. Mo-mediated loss of DG function caused disruption of the basal lamina layers, increased apoptosis and reduction of the expression domains of specific retinal markers, at early stages. Later in development, morphants displayed unilateral ocular malformations, such as microphtalmia and retinal delayering with photoreceptors and ganglion cells scattered throughout the retina or aggregated in rosette-like structures. These results recall the phenotypes observed in specific human diseases and suggest that DG presence is crucial at early stages for the organization of retinal architecture.
肌营养不良聚糖（DG）是一种将细胞外基质与内部细胞骨架连接起来的跨膜受体。其结构功能主要在肌纤维中得到表征，但DG在不同组织和细胞类型中也发挥着信号传导和发育作用。我们研究了非洲爪蟾眼睛发育过程中肌营养不良聚糖缺失的影响。我们在四细胞期胚胎的一个背侧卵裂球的动物极注射了一种特异性吗啉代（Mo）反义寡核苷酸。Mo介导的DG功能丧失在早期导致基底层破坏、细胞凋亡增加以及特定视网膜标志物表达域减少。在发育后期，形态异常的胚胎表现出单侧眼部畸形，如小眼球症和视网膜分层紊乱，光感受器和神经节细胞散布在整个视网膜中或聚集形成玫瑰花结样结构。这些结果使人联想到在特定人类疾病中观察到的表型，并表明DG的存在在视网膜结构组织的早期阶段至关重要。
