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Abstract
Inherited retinal degenerations (IRDs) cause permanent vision impairment or vision loss due to the death of rod and cone photoreceptors. Animal models of IRDs have been instrumental in providing knowledge of the pathological mechanisms that cause photoreceptor death and in developing successful approaches that could slow or prevent vision loss. Zebrafish models of IRDs represent an ideal model system to study IRDs in a cone-rich retina and to test strategies that exploit the natural ability to regenerate damaged neurons. This review highlights those zebrafish mutants and transgenic lines that exhibit adult-onset retinal degeneration and serve as models of retinitis pigmentosa, cone-rod dystrophy, and ciliopathies.
Highlights
Inherited retinal degenerations (IRDs) comprise a large collection of diseases that progressively compromise vision and can lead to blindness due to the degeneration of rod and cone photoreceptors
retinitis pigmentosa (RP) is associated with several syndromic and systemic conditions, where the disease impacts one or more other organs. These diseases can include Usher syndrome, in which RP is associated with hearing loss, Joubert syndrome (JBTS) and Bardet-Biedl syndrome (BBS), which are two ciliopathies with systemic effects, and Senior-Loken syndrome, which presents with RP and kidney disease
The purpose of this review is to examine those zebrafish mutants that are adultviable and exhibit progressive retinal degeneration
Summary
Inherited retinal degenerations (IRDs) comprise a large collection of diseases that progressively compromise vision and can lead to blindness due to the degeneration of rod and cone photoreceptors. Tg(Xla.Rho:GAP-CFP)q13Tg The first report of a model with rod-specific degeneration described a transgenic line of zebrafish that expresses a membrane-targeted cyan fluorescent protein (mCFP) using a 5.5 kb promoter sequence from the Xenopus laevis rhodopsin gene[130,131]. Tg(rho:Mmu.Rho_P23H-FLAG)uth4Tg Transgenic models of adRP that express mutant forms of rhodopsin in rod photoreceptors have been made in mice[141,142,143], rats[144], and frogs[145,146,147,148] These models exhibit progressive rod degeneration and have proven invaluable toward understanding the mechanisms of degeneration caused by different pathogenic mutations in the rhodopsin gene. This differs from the rhodopsin (rh1-1) knockout zebrafish mutants, which had normal cone morphology[42] At both 4 mpf and 6 mpf, it was noticed that the number of proliferating cells (PCNA+) was considerably higher in the ONL of transgenic animals compared to wild-type. Understanding potential differences in the transcriptional responses of Müller glia[155] and microglia[156] to disease and injury will hopefully help answer why zebrafish do not regenerate in these IRD models
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