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颗粒蛋白前体缺乏导致发育过程中的视网膜神经节细胞丢失。

Progranulin deficiency causes the retinal ganglion cell loss during development.

机构信息

Molecular Pharmacology, Department of Biofunctional Evaluation, Gifu Pharmaceutical University, 1-25-4 Daigaku-nishi, Gifu, 501-1196, Japan.

出版信息

Sci Rep. 2017 May 10;7(1):1679. doi: 10.1038/s41598-017-01933-8.

Abstract

Astrocytes are glial cells that support and protect neurons in the central nervous systems including the retina. Retinal ganglion cells (RGCs) are in contact with the astrocytes and our earlier findings showed the reduction of the number of cells in the ganglion cell layer in adult progranulin deficient mice. In the present study, we focused on the time of activation of the astrocytes and the alterations in the number of RGCs in the retina and optic nerve in progranulin deficient mice. Our findings showed that the number of Brn3a-positive cells was reduced and the expression of glial fibrillary acidic protein (GFAP) was increased in progranulin deficient mice. The progranulin deficient mice had a high expression of GFAP on postnatal day 9 (P9) but not on postnatal day 1. These mice also had a decrease in the number of the Brn3a-positive cells on P9. Taken together, these findings indicate that the absence of progranulin can affect the survival of RGCs subsequent the activation of astrocytes during retinal development.

摘要

星形胶质细胞是中枢神经系统(包括视网膜)中支持和保护神经元的神经胶质细胞。视网膜神经节细胞(RGC)与星形胶质细胞接触,我们之前的研究结果表明,在成年颗粒蛋白缺乏的小鼠中,神经节细胞层中的细胞数量减少。在本研究中,我们专注于星形胶质细胞的激活时间以及颗粒蛋白缺乏的小鼠视网膜和视神经中 RGC 数量的变化。我们的研究结果表明,颗粒蛋白缺乏的小鼠中 Brn3a 阳性细胞的数量减少,神经胶质纤维酸性蛋白(GFAP)的表达增加。颗粒蛋白缺乏的小鼠在出生后第 9 天(P9)GFAP 表达水平较高,但在出生后第 1 天没有表达。这些小鼠在 P9 时 Brn3a 阳性细胞的数量也减少了。综上所述,这些发现表明,颗粒蛋白缺乏会影响视网膜发育过程中星形胶质细胞激活后 RGC 的存活。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fcc4/5431873/45fd6416ef82/41598_2017_1933_Fig1_HTML.jpg

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