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首页> 外文期刊>Biology Open >A novel immunodeficient NOD.SCID-rd1?mouse model of retinitis pigmentosa to investigate potential therapeutics and pathogenesis of retinal degeneration
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A novel immunodeficient NOD.SCID-rd1?mouse model of retinitis pigmentosa to investigate potential therapeutics and pathogenesis of retinal degeneration

机译：一种新型的视网膜色素变性免疫缺陷型NOD.SCID-rd1？小鼠模型，用于研究视网膜变性的潜在治疗方法和发病机理

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Retinitis pigmentosa (RP) is a common retinal degeneration disease caused by mutation in any gene of the photo transduction cascade and results in photoreceptor dystrophy. Over decades, several animal models have been used to address the need for the elucidation of effective therapeutics and factors regulating retinal degeneration to prohibit or renew the damaged retina. However, controversies over the immune privilege of retina during cell transplantation and the role of immune modulation during RP still remain largely uninvestigated because of the lack of suitable animal models. Here, we have developed an immunocompromised mouse model, NOD.SCID-rd1, for retinitis pigmentosa (RP) by crossing CBA/J and NOD SCID mice and selecting homozygous double mutant animals for further breeding. Characterization of the newly developed RP model indicates a similar retinal degeneration pattern as CBA/J, with a decreased apoptosis rate and rhodopsin loss. It also exhibits loss of T cells, B cells and NK cells. The NOD.SCID-rd1?model is extremely useful for allogenic and xenogenic cell-based therapeutics, as indicated by the higher cell integration capacity post transplantation. We dissect the underlying role of the immune system in the progression of RP and the effect of immune deficiency on immune privilege of the eye using comparative qPCR studies of this model and the immune-competent RP model.
机译：色素性视网膜炎（RP）是一种常见的视网膜变性疾病，由光转导级联的任何基因突变引起，并导致光感受器营养不良。几十年来，已经使用了几种动物模型来解决阐明有效的治疗方法和调节视网膜变性以阻止或更新受损视网膜的因素的需求。然而，由于缺乏合适的动物模型，关于在细胞移植过程中视网膜免疫特权的争议以及在RP期间免疫调节的作用仍未得到充分研究。在这里，我们通过跨CBA / J和NOD SCID小鼠并选择纯合双突变动物进行进一步育种，为色素性视网膜炎（RP）开发了免疫受损的小鼠模型NOD.SCID-rd1。新开发的RP模型的特征表明与CBA / J相似的视网膜变性模式，具有降低的细胞凋亡率和视紫红质损失。它还表现出T细胞，B细胞和NK细胞的丢失。 NOD.SCID-rd1α模型对于同种异体和异种细胞为基础的治疗非常有用，移植后具有更高的细胞整合能力。我们使用该模型和具有免疫功能的RP模型的比较qPCR研究，剖析了免疫系统在RP进程中的潜在作用以及免疫缺陷对眼睛免疫特权的影响。

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