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首页> 外文期刊>Scientific reports. >The bacterial toxin CNF1 as a tool to induce retinal degeneration reminiscent of retinitis pigmentosa
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The bacterial toxin CNF1 as a tool to induce retinal degeneration reminiscent of retinitis pigmentosa

机译：细菌毒素CNF1作为诱导视网膜变性的工具让人让人联想过敏性炎症

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Retinitis pigmentosa (RP) comprises a group of inherited pathologies characterized by progressive photoreceptor degeneration. In rodent models of RP, expression of defective genes and retinal degeneration usually manifest during the first weeks of postnatal life, making it difficult to distinguish consequences of primary genetic defects from abnormalities in retinal development. Moreover, mouse eyes are small and not always adequate to test pharmacological and surgical treatments. An inducible paradigm of retinal degeneration potentially extensible to large animals is therefore desirable. Starting from the serendipitous observation that intraocular injections of a Rho GTPase activator, the bacterial toxin Cytotoxic Necrotizing Factor 1 (CNF1), lead to retinal degeneration, we implemented an inducible model recapitulating most of the key features of Retinitis Pigmentosa. The model also unmasks an intrinsic vulnerability of photoreceptors to the mechanism of CNF1 action, indicating still unexplored molecular pathways potentially leading to the death of these cells in inherited forms of retinal degeneration.
机译：视网膜炎PIGMENTOSA（RP）包含一组遗传性病理，其特征在于渐进式光感受器变性。在RP的啮齿动物模型中，在出生后生命的第一周，缺陷基因和视网膜变性的表达通常显现，使得难以区分原遗传缺陷的后果免受视网膜发育的异常。此外，小鼠的眼睛很小，并不总是足以测试药理学和手术治疗。因此，希望潜在地伸展对大型动物的视网膜变性的诱导范例。从偶发观察开始，即rho GTP酶活激活物的眼内注射，细菌毒素细胞毒性坏死因子1（CNF1）导致视网膜变性，我们实施了一种诱导型模型，重新携带了视网膜炎色素的大部分关键特征。该模型还使光感受器的内在脆性对CNF1作用的机制，表明仍然是未探测的分子途径，可能导致这些细胞在继承的视网膜变性中的死亡。

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