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中華民國視網膜色素病變協會
:::左側區塊

各位會員大家好

發佈日期:2018/03/30

內容:
各位會員大家好,春假即將來臨在此祝福大家春假愉快,分享一篇與抗氧化劑的相關研究,有在服用協會抗氧化劑的會員們,記得服用期間要配合運動喔~以下研究為針對視網膜色素病變中的尤賽斯綜合症,研究中指出錐細胞病變是由缺乏USH1蛋白而觸發,但可藉由抗氧化治療來預防。尤賽斯綜合症造成失去桿細胞後的二次錐細胞病變,而視網膜病變的治療受到阻礙是因為在缺乏Ush1基因的突變小鼠中找不到視網膜病變的明確證據,但卻會造成聽力的退化。研究者發現,當突變小鼠飼養在黑暗中和補充抗氧化劑時可以預防感光細胞凋亡及視網膜的退化。

5. Sci Rep. 2018 Jan 31;8(1):1968. doi: 10.1038/s41598-018-20171-0.
Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments.
Trouillet A(1), Dubus E(1), Dégardin J(1), Estivalet A(1), Ivkovic I(1), Godefroy D(1), García-Ayuso D(2), Simonutti M(1), Sahly I(3)(4), Sahel JA(1)(5)(6)(7),El-Amraoui A(3)(4), Petit C(3)(4)(8), Picaud S(9).
Author information:
(1)Sorbonne Université, INSERM, CNRS, Institut de la Vision, 17 rue Moreau, F-75012, Paris, France.
(2)Departamento de Oftalmología, Facultad de Medicina, Universidad de Murcia, and Instituto Murciano de Investigación Biosanitaria- Hospital Virgen de la Arrixaca (IMIB-Arrixaca), 30100, Murcia, Spain.
(3)Génétique et Physiologie de l`Audition, Institut Pasteur, 75015, Paris, France.
(4)UMRS 1120, Institut National de la Santé et de la Recherche Médicale, 75015, Paris, France.
(5)CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DGOS CIC 1423, 28 rue de Charenton, F-75012, Paris, France.
(6)Fondation Ophtalmologique Adolphe de Rothschild, 75019, Paris, France.
(7)Académie des Sciences, 75006, Paris, France.
(8)Collège de France, 75005, Paris, France.
(9)Sorbonne Université, INSERM, CNRS, Institut de la Vision, 17 rue Moreau, F-75012, Paris, France. serge.picaud@inserm.fr.
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.
DOI: 10.1038/s41598-018-20171-0
PMID: 29386551