Article
Usherin defects lead to early-onset retinal dysfunction in zebrafish.
Experimental eye research - 1 Aug 2018
Dona Margo, Slijkerman Ralph, Lerner Kimberly, Broekman Sanne, Wegner Jeremy, Howat Taylor, Peters Theo, Hetterschijt Lisette, Boon Nanda, de Vrieze Erik, Sorusch Nasrin, Wolfrum Uwe, Kremer Hannie, Neuhauss Stephan, Zang Jingjing, Kamermans Maarten, Westerfield Monte, Phillips Jennifer, van Wijk Erwin
Abstract excerpt
Mutations in USH2A are the most frequent cause of Usher syndrome and autosomal recessive nonsyndromic retinitis pigmentosa. To unravel the pathogenic mechanisms underlying USH2A-associated retinal degeneration and to evaluate future therapeutic strategies that could potentially halt the progression of this devastating disorder, an animal model is needed. The available Ush2a knock-out mouse model does not mimic...
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