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首页> 外文期刊>Investigative ophthalmology & visual science >Heterozygous Pitx2 Null Mice Accurately Recapitulate the Ocular Features of Axenfeld-Rieger Syndrome and Congenital Glaucoma
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Heterozygous Pitx2 Null Mice Accurately Recapitulate the Ocular Features of Axenfeld-Rieger Syndrome and Congenital Glaucoma

机译：杂合Pitx2空小鼠准确地概括了Axenfeld-Rieger综合征和先天性青光眼的眼部特征

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Purpose: The purpose of this analysis was to assess the utility of Pitx2+/a?? mice as a model for the ocular features of Axenfeld-Rieger Syndrome and for congenital glaucoma. Methods: Eyes of Pitx2+/a?? and wild-type littermates were examined clinically using optical coherence tomography (OCT) and fundus photography. Intraocular pressures were measured using a TonoLab rebound tonometer. Eyes were examined histologically to assess PITX2 expression, structural integrity, and optic nerve and ganglion cell content. Results: PITX2 is present postnatally in the corneal endothelium and stroma, iris stroma, trabecular meshwork, and Schlemm's canal. Reduced central corneal thickness, iris defects, and iridicorneal adhesions are all prevalent in Pitx2+/a?? eyes. Although optic nerve heads appear normal at postnatal day 7, IOP is elevated and optic nerve head cupping is fully penetrant in Pitx2+/a?? eyes by 3 weeks of age. Neurodegeneration is present in a significant percentage of optic nerves from Pitx2+/a?? mice by 3 weeks of age, and is fully penetrant by 2 months of age. Pitx2+/a?? eyes show significant reductions in specifically ganglion cell density in all four quadrants by 2 months of age. Conclusions: Pitx2+/a?? mice model the major ocular features of Axenfeld-Rieger Syndrome and will be an important resource for understanding the molecular mechanisms leading to anterior segment dysgenesis and a high prevalence of glaucoma in this disease. In addition, these mice may provide an efficient new model for assessing the molecular events in glaucoma more generally, and for developing and testing new treatment paradigms for this disease.
机译：目的：该分析的目的是评估Pitx2 + / a？小鼠作为Axenfeld-Rieger综合征的眼部特征和先天性青光眼的模型。方法：Pitx2 + / a的眼睛？临床上使用光学相干断层扫描（OCT）和眼底照相检查野生型和同窝仔。使用TonoLab回弹眼压计测量眼内压。对眼睛进行组织学检查，以评估PITX2表达，结构完整性以及视神经和神经节细胞含量。结果：PITX2在出生后存在于角膜内皮和间质，虹膜基质，小梁网和施莱姆管中。减少的中央角膜厚度，虹膜缺损和虹膜角膜粘连在Pitx2 + / a？眼睛。尽管在出生后第7天视神经头看起来正常，但Pitx2 + / a？中的IOP升高且视神经头拔罐完全渗透。 3周大的眼睛。 Pitx2 + / a？视神经中有很大一部分存在神经变性。 3周龄的老鼠，到2月龄完全渗透。 Pitx2 + / a ??眼睛显示，到2个月大时，所有四个象限的神经节细胞密度均明显降低。结论：Pitx2 + / a ??小鼠模拟了Axenfeld-Rieger综合征的主要眼部特征，将成为了解导致该疾病前节发育不良和青光眼高发的分子机制的重要资源。此外，这些小鼠可提供一种有效的新模型，用于更广泛地评估青光眼中的分子事件，以及开发和测试该疾病的新治疗范例。

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