Inactivation of the microRNA-183/96/182 cluster results in syndromic retinal degeneration
| Autor: | Shunbin Xu, Caroline E. Haldin, Daniel A. Nicholson, Donald J. Zack, Peter E. Larsen, Karl J. Wahlin, Colleen Cowan, Sanja Turturro, David Valle, Stephen Lumayag, Beatrix Kovacs, P. Dane Witmer, Nicola J. Corbett |
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| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: |
Retinal degeneration
genetic structures Light Neurogenesis Molecular Sequence Data Synaptogenesis Biology Neurotransmission Synaptic Transmission Retina chemistry.chemical_compound Mice medicine Journal Article Animals Vision Ocular Mice Knockout Multidisciplinary Research Support Non-U.S. Gov't Retinal Degeneration Sense Organs Retinal Syndrome medicine.disease Introns Disease Models Animal MicroRNAs medicine.anatomical_structure PNAS Plus chemistry Multigene Family Knockout mouse Synapses sense organs Neuroscience Visual phototransduction Photoreceptor Cells Vertebrate |
| Zdroj: | Lumayag, S, Haldin, C E, Corbett, N J, Wahlin, K J, Cowan, C, Turturro, S, Larsen, P E, Kovacs, B, Witmer, P D, Valle, D, Zack, D J, Nicholson, D A & Xu, S 2013, ' Inactivation of the microRNA-183/96/182 cluster results in syndromic retinal degeneration ', Proceedings of the National Academy of Sciences of the United States of America, vol. 110, no. 6, pp. E507-E516 . https://doi.org/10.1073/pnas.1212655110 |
| Popis: | The microRNA -183/96/182 cluster 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-183C GT/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 decreased b -wave amplitude as the primary defect and progressive retinal degeneration. In addition, inactivation of the miR-183/96/182 cluster resulted in global changes in retinal gene expression, with enrichment of genes important for synaptogenesis, synaptic transmission, photoreceptor morphogenesis, and phototransduction, suggesting that the miR-183/96/182 cluster plays important roles in postnatal functional differentiation and synaptic connectivity of photoreceptors. |
| Databáze: | OpenAIRE |
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