Deletion of prominin-1 in mice results in disrupted photoreceptor outer segment protein homeostasis
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Xiao-Dong Sun and Xue-Ting Luo. Department of Ophthalmology, Shanghai First People’s Hospital, Shanghai Jiao Tong University School of Medicine, 100 Hai Ning Road, Shanghai 200080, China. xdsun@sjtu.edu.cn; xtluo@sjtu.edu.cn

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Supported by the National Natural Science Foundation of China (No.81730026); the National Key R&D Program (No.2017YFA0105301; No.2019ZX09301113); the Science and Technology Commission of Shanghai Municipality (No.17411953000).

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    Abstract:

    AIM: To illustrate the underlying mechanism how prominin-1 (also known as Prom1) mutation contribute to progressive photoreceptor degeneration. METHODS: A CRISPR-mediated Prom1 knockout (Prom1-KO) mice model in the C57BL/6 was generated and the photoreceptor degeneration phenotypes by means of structural and functional tests were demonstrated. Immunohistochemistry and immunoblot analysis were performed to reveal the localization and quantity of related outer segment (OS) proteins. RESULTS: The Prom1-KO mice developed the photoreceptor degeneration phenotype including the decreased outer nuclear layer (ONL) thickness and compromised electroretinogram amplitude. Immunohistochemistry analysis revealed impaired trafficking of photoreceptor OS proteins. Immunoblot data demonstrated decreased photoreceptor OS proteins. CONCLUSION: Prom1 deprivation causes progressive photoreceptor degeneration. Prom1 is essential for maintaining normal trafficking and normal quantity of photoreceptor OS proteins. The new light is shed on the pathogenic mechanism underlying photoreceptor degeneration caused by Prom1 mutation.

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Yu-Shu Xiao, Jian Liang, Min Gao, et al. Deletion of prominin-1 in mice results in disrupted photoreceptor outer segment protein homeostasis. Int J Ophthalmol, 2021,14(9):1334-1344

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Publication History
  • Received:January 28,2021
  • Revised:April 21,2021
  • Adopted:
  • Online: August 23,2021
  • Published: