Inactivation of the microRNA-183/96/182cluster results in syndromic retinal degeneration
Copyright policy )Inactivation of the microRNA-183/96/182cluster results in syndromic retinal degeneration
The microRNA-183/96/182cluster is highly expressed in the retina and other sensory organs. To uncover its in vivo functions in the retina, we generated a knockout mouse model, designated “miR-183CGT/GT,” using a gene-trap embryonic stem cell clone. We provide evidence that inactivation of the cluster results in early-onset and progressive synaptic defects of the photoreceptors, leading to abnormalities of scotopic and photopic electroretinograms with decreasedb-wave amplitude as the primary defect and progressive retinal degeneration. In addition, inactivation of themiR-183/96/182cluster resulted in global changes in retinal gene expression, with enrichment of genes important for synaptogenesis, synaptic transmission, photoreceptor morphogenesis, and phototransduction, suggesting that themiR-183/96/182cluster plays important roles in postnatal functional differentiation and synaptic connectivity of photoreceptors.
- Johns Hopkins Medicine United States
- University of Salford United Kingdom
- Argonne National Laboratory United States
- Johns Hopkins University United States
- Rush University Medical Center United States
Light, Vision, Knockout, Neurogenesis, Molecular Sequence Data, Research Support, Synaptic Transmission, Retina, Mice, Ocular, Journal Article, Animals, Photoreceptor Cells, Non-U.S. Gov't, Vision, Ocular, Mice, Knockout, Animal, Vertebrate, Research Support, Non-U.S. Gov't, Retinal Degeneration, Sense Organs, Syndrome, Introns, Disease Models, Animal, MicroRNAs, Multigene Family, Disease Models, Synapses, Photoreceptor Cells, Vertebrate
Light, Vision, Knockout, Neurogenesis, Molecular Sequence Data, Research Support, Synaptic Transmission, Retina, Mice, Ocular, Journal Article, Animals, Photoreceptor Cells, Non-U.S. Gov't, Vision, Ocular, Mice, Knockout, Animal, Vertebrate, Research Support, Non-U.S. Gov't, Retinal Degeneration, Sense Organs, Syndrome, Introns, Disease Models, Animal, MicroRNAs, Multigene Family, Disease Models, Synapses, Photoreceptor Cells, Vertebrate
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