Inactivation of the microRNA-183/96/182 cluster results in syndromic retinal degeneration

Stephen Lumayag, Caroline E. Haldin, Nicola J. Corbett, Karl J. Wahlin, Colleen Cowan, Sanja Turturro, Peter E. Larsen, Beatrix Kovacs, P. Dane Witmer, David Valle, Donald J. Zack, Daniel A. Nicholson, Shunbin Xu

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128 Scopus citations


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- 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 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, photoreceptormorphogenesis, and phototransduction, suggesting that the miR-183/96/182 cluster plays important roles in postnatal functional differentiation and synaptic connectivity of photoreceptors.

Original languageEnglish (US)
Pages (from-to)E507-E516
JournalProceedings of the National Academy of Sciences of the United States of America
Issue number6
StatePublished - Feb 5 2013

ASJC Scopus subject areas

  • General


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