ArticleExperimental eye research2014
Photoreceptors in whirler mice show defective transducin translocation and are susceptible to short-term light/dark changes-induced degeneration.
Article in Experimental eye research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 23 citations in OpenAlex.
- Transcriptional downregulation of rhodopsin is associated with desensitization of rods to light-induced damage in a murine model of retinitis pigmentosa.Human molecular genetics · 2025Article
- Successful large gene augmentation of USH2A with non-viral episomal vectors.Molecular therapy : the journal of the American Society of Gene Therapy · 2023Article
- The usherin mutation c.2299delG leads to its mislocalization and disrupts interactions with whirlin and VLGR1.Nature communications · 2023Article
- USH2A Gene Mutations in Rabbits Lead to Progressive Retinal Degeneration and Hearing Loss.Translational vision science & technology · 2023Article
- Clinical and preclinical therapeutic outcome metrics for USH2A-related disease.Human molecular genetics · 2020Article
- Review
- Transducin Partners Outside the Phototransduction Pathway.Frontiers in cellular neuroscience · 2020Article
- Cul3-Klhl18 ubiquitin ligase modulates rod transducin translocation during light-dark adaptation.The EMBO journal · 2019Article
- Usher syndrome and non-syndromic deafness: Functions of different whirlin isoforms in the cochlea, vestibular organs, and retina.Hearing research · 2019Review
- Clinical applications of retinal gene therapies.Precision clinical medicine · 2018Review
- Integrin α8 and Pcdh15 act as a complex to regulate cilia biogenesis in sensory cells.Journal of cell science · 2017Article
- AAV-Mediated Clarin-1 Expression in the Mouse Retina: Implications for USH3A Gene Therapy.PloS one · 2016Article
- Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities.Biochimica et biophysica acta · 2015Review
- EIAV-based retinal gene therapy in the shaker1 mouse model for usher syndrome type 1B: development of UshStat.PloS one · 2014Article
- Article
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
Abstract
Usher syndrome combines congenital hearing loss and retinitis pigmentosa (RP). Mutations in the whirlin gene (DFNB31/WHRN) cause a subtype of Usher syndrome (USH2D). Whirler mice have a defective whirlin gene. They have inner ear defects but usually do not develop retinal degeneration. Here we report that, in whirler mouse photoreceptors, the light-activated rod transducin translocation is delayed and its activation threshold is shifted to a higher level. Rhodopsin mis-localization is observed in rod inner segments. Continuous moderate light exposure can induce significant rod photoreceptor degeneration. Whirler mice reared under a 1500 lux light/dark cycle also develop severe photoreceptor degeneration. Previously, we have reported that shaker1 mice, a USH1B model, show moderate light-induced photoreceptor degeneration with delayed transducin translocation. Here, we further show that, in both whirler and shaker1 mice, short-term moderate light/dark changes can induce rod degeneration as severe as that induced by continuous light exposure. The results from shaker1 and whirler mice suggest that defective transducin translocation may be functionally related to light-induced degeneration, and these two symptoms may be caused by defects in Usher protein function in rods. Furthermore, these results indicate that both Usher syndrome mouse models possess a light-induced retinal phenotype and may share a closely related pathobiological mechanism.
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