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BXD32小鼠:慢性视网膜炎症和光感受器退化的高保真模型。

The BXD32 Mouse: A High-Fidelity Model of Chronic Retinal Inflammation and Photoreceptor Degeneration.

作者信息

Hollingsworth T J, Meshkat Bahar, Wang Xiangdi, White William A, Marquez-Wilkins Esther, Jablonski Monica M

机构信息

Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, TN, USA.

Department of Anatomy and Neurobiology, University of Tennessee Health Science Center, Memphis, TN, USA.

出版信息

Adv Exp Med Biol. 2025;1468:45-50. doi: 10.1007/978-3-031-76550-6_8.

DOI:10.1007/978-3-031-76550-6_8
PMID:39930171
Abstract

The study of retinal degenerations (RDs) is a field involving all aspects of retinal anatomy, physiology, and genetics. RDs are heterogeneous involving many genes and systems underlying their pathogeneses. To understand these processes, animal models act as surrogates for human studies; however, an ever-existent issue is few models offer high-fidelity and direct correlations to the human condition. Our recent work has established an animal model that is able to fill both needs. The BXD32 mouse exhibits a polygenic inherited retinal dystrophy (IRD) that correlates to human disease through aberrant disc formation and chronic retinal inflammation working in concert with the genetic underliers to advance the disease. This model can serve to test anti-inflammatory treatments directly to affected tissue avoiding systemic issues as well as understanding the pathophysiology of human IRDs.

摘要

视网膜变性(RDs)的研究是一个涉及视网膜解剖学、生理学和遗传学各个方面的领域。RDs具有异质性,其发病机制涉及许多基因和系统。为了理解这些过程,动物模型充当人类研究的替代物;然而,一个一直存在的问题是,很少有模型能与人类疾病有高度保真度和直接的相关性。我们最近的工作建立了一种能够满足这两个需求的动物模型。BXD32小鼠表现出一种多基因遗传性视网膜营养不良(IRD),它通过异常的视盘形成和慢性视网膜炎症与遗传因素协同作用导致疾病,从而与人类疾病相关。该模型可用于直接对受影响组织进行抗炎治疗测试,避免全身问题,同时有助于理解人类IRD的病理生理学。

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本文引用的文献

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Stargardt's pigmentosa: A novel combination of two inherited retinal dystrophies.斯特格眼病:两种遗传性视网膜病变的新型组合。
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Cellular and Molecular Mechanisms of Pathogenesis Underlying Inherited Retinal Dystrophies.遗传性视网膜疾病发病机制的细胞和分子机制。
Biomolecules. 2023 Feb 1;13(2):271. doi: 10.3390/biom13020271.
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Targeted P2X7/NLRP3 signaling pathway against inflammation, apoptosis, and pyroptosis of retinal endothelial cells in diabetic retinopathy.
靶向 P2X7/NLRP3 信号通路抑制糖尿病视网膜病变中视网膜内皮细胞的炎症、凋亡和焦亡。
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Chronic Proinflammatory Signaling Accelerates the Rate of Degeneration in a Spontaneous Polygenic Model of Inherited Retinal Dystrophy.慢性促炎信号加速遗传性视网膜营养不良自发多基因模型中的退化速度。
Front Pharmacol. 2022 Mar 21;13:839424. doi: 10.3389/fphar.2022.839424. eCollection 2022.
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Inherited retinal diseases: Linking genes, disease-causing variants, and relevant therapeutic modalities.遗传性视网膜疾病:基因、致病变异与相关治疗方式的关联。
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Anti-inflammatory and neuroprotective properties of the corticosteroid fludrocortisone in retinal degeneration.氟氢可的松在视网膜变性中的抗炎和神经保护作用。
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Proinflammatory Pathways Are Activated in the Human Q344X Rhodopsin Knock-In Mouse Model of Retinitis Pigmentosa.促炎途径在人类 Q344X 视蛋白敲入型色素性视网膜炎小鼠模型中被激活。
Biomolecules. 2021 Aug 6;11(8):1163. doi: 10.3390/biom11081163.
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Cell Syst. 2021 Mar 17;12(3):235-247.e9. doi: 10.1016/j.cels.2020.12.002. Epub 2021 Jan 19.
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