title: Dystroglycan is required for proper retinal layering.
abstract: 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.
authors.affiliation: Dipartimento di Fisiologia e Biochimica, Laboratori di Biologia Cellulare e dello Sviluppo, Università di Pisa, via G. Carducci 13, Ghezzano, Pisa 56010, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Lunardi
authors.forename: Federico
authors.initials: F
authors.lastname: Cremisi
authors.forename: Luciana
authors.initials: L
authors.lastname: Dente
mesh_terms: D000818:Animals; D019521:Body Patterning; D001973:Bromodeoxyuridine / Q000494:pharmacology; D003599:Cytoskeleton / Q000378:metabolism; D049030:Dystroglycans / Q000096:biosynthesis / Q000378:metabolism / Q000502:physiology*; D005260:Female; D018507:Gene Expression Regulation, Developmental; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D020287:In Situ Nick-End Labeling; D051739:Microphthalmia-Associated Transcription Factor / Q000378:metabolism; D016376:Oligonucleotides, Antisense / Q000494:pharmacology; D010641:Phenotype; D010786:Photoreceptor Cells / Q000196:embryology; D012160:Retina / Q000196:embryology*; D012165:Retinal Ganglion Cells / Q000378:metabolism; D014018:Tissue Distribution; D014981:Xenopus; D014982:Xenopus laevis
chemical_list: D051739:Microphthalmia-Associated Transcription Factor; D016376:Oligonucleotides, Antisense; D049030:Dystroglycans; D001973:Bromodeoxyuridine