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klotho基因敲除突变导致视网膜变性,其特征为功能障碍、胶质细胞增生以及β淀粉样蛋白和过度磷酸化tau蛋白的沉积。

Klotho null mutation leads to retinal degeneration characterized by functional impairment, gliosis, and deposition of amyloid-beta and hyperphosphorylated tau proteins.

作者信息

Chen Zhi-Jia, Wu Chun-Yen, Hsiao Fang-Yan, Ma Jing-Chun, Lai Gabriel, Chang Han-Hsin, Lin David Pei-Cheng

机构信息

Department of Medical Laboratory and Biotechnology, Chung Shan Medical University, Taichung, TaiwanRepublic of China.

Department of Nutrition, Chung Shan Medical University, Taichung, TaiwanRepublic of China.

出版信息

PLoS One. 2025 May 15;20(5):e0323633. doi: 10.1371/journal.pone.0323633. eCollection 2025.

Abstract

BACKGROUND

Klotho mutation has been known to accelerate aging and degenerative pathogenesis, notably in the kidney and the brain. Nevertheless, the aftermath of Klotho function deprivation in the retina has not been detailed. This study aimed to provide an in-depth analysis of retinopathy caused by Klotho mutation.

RESULTS

The homozygous Klotho mutant mice retinas were analyzed at the 6th, 8th and 10th weeks of age, along with their heterozygous, and wild-type littermates between both genders. The electroretinogram results showed retinal function impairment with Klotho null mutation, as compared with their littermates. Nevertheless, there was no difference in the retinal layer thickness, morphology, and cell death up to 10 weeks of age among the three genotypes. No evident damage was detected in the photoreceptors, interneurons, and retinal ganglion cells with Klotho mutation up to 10 weeks of age. Amyloid-beta and hyperphosphorylated tau protein deposits were detected in the Klotho mutant retina, along with glial cell activation at 10 weeks of age.

CONCLUSIONS

The results revealed that Klotho null mutation leads to retinal degeneration characterized by functional impairments, gliosis, and amyloid-beta and hyperphosphorylated tau protein deposition in the retina. Klotho protein function is, therefore, mandatory for the maintenance of a healthy retina. The mouse Klotho null mutation may be used as a study model for age-related retinal degeneration and Alzheimer's disease.

摘要

背景

已知klotho突变会加速衰老和退行性病变进程,尤其是在肾脏和大脑中。然而,视网膜中klotho功能缺失的后果尚未详细阐明。本研究旨在深入分析由klotho突变引起的视网膜病变。

结果

在6周龄、8周龄和10周龄时对纯合klotho突变小鼠的视网膜进行分析,并与它们的杂合子和野生型同窝小鼠进行比较,涵盖两种性别。视网膜电图结果显示,与同窝小鼠相比,klotho基因敲除突变导致视网膜功能受损。然而,在10周龄之前,三种基因型小鼠的视网膜层厚度、形态和细胞死亡情况并无差异。在10周龄之前,未检测到klotho突变小鼠的光感受器、中间神经元和视网膜神经节细胞有明显损伤。在10周龄时,在klotho突变小鼠的视网膜中检测到β淀粉样蛋白和过度磷酸化的tau蛋白沉积,同时伴有胶质细胞活化。

结论

结果表明,klotho基因敲除突变导致视网膜变性,其特征为视网膜功能障碍、胶质细胞增生以及β淀粉样蛋白和过度磷酸化的tau蛋白沉积。因此,klotho蛋白功能对于维持健康的视网膜至关重要。小鼠klotho基因敲除突变可作为与年龄相关的视网膜变性和阿尔茨海默病的研究模型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd6e/12080808/fb9d6e549667/pone.0323633.g001.jpg

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