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© Research
Publication : Scientific reports

Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments

Scientific Fields
Diseases
Organisms
Applications
Technique

Published in Scientific reports - 31 Jan 2018

Trouillet A, Dubus E, Dégardin J, Estivalet A, Ivkovic I, Godefroy D, García-Ayuso D, Simonutti M, Sahly I, Sahel JA, El-Amraoui A, Petit C, Picaud S

Link to Pubmed [PMID] – 29386551

Sci Rep 2018 Jan;8(1):1968

Usher syndrome type 1 (USH1) is a major cause of inherited deafness and blindness in humans. The eye disorder is often referred to as retinitis pigmentosa, which is characterized by a secondary cone degeneration following the rod loss. The development of treatments to prevent retinal degeneration has been hampered by the lack of clear evidence for retinal degeneration in mutant mice deficient for the Ush1 genes, which instead faithfully mimic the hearing deficit. We show that, under normal housing conditions, Ush1g and Ush1c albino mice have dysfunctional cone photoreceptors whereas pigmented knockout animals have normal photoreceptors. The key involvement of oxidative stress in photoreceptor apoptosis and the ensued retinal gliosis were further confirmed by their prevention when the mutant mice are reared under darkness and/or supplemented with antioxidants. The primary degeneration of cone photoreceptors contrasts with the typical forms of retinitis pigmentosa. Altogether, we propose that oxidative stress probably accounts for the high clinical heterogeneity among USH1 siblings, which also unveils potential targets for blindness prevention.

https://www.ncbi.nlm.nih.gov/pubmed/29386551