Article
Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments.
Scientific reports - 31 Jan 2018
Trouillet Alix, Dubus Elisabeth, Dégardin Julie, Estivalet Amrit, Ivkovic Ivana, Godefroy David, García-Ayuso Diego, Simonutti Manuel, Sahly Iman, Sahel José A, El-Amraoui Aziz, Petit Christine, Picaud Serge
Abstract excerpt
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,...
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