Heterozygous Pitx2 Null Mice Accurately Recapitulate the Ocular Features of Axenfeld-Rieger Syndrome and Congenital Glaucoma.
Heterozygous Pitx2 Null Mice Accurately Recapitulate the Ocular Features of Axenfeld-Rieger Syndrome and Congenital Glaucoma.
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DOI：
10.1167/iovs.16-19700
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发表时间：
2016-09-01
影响因子：
4.4
通讯作者：
Gage PJ
中科院分区：
文献类型：
--
作者：
Chen L;Gage PJ
该分析的目的是评估Pitx 2 +/−小鼠作为Axenet-Rieger综合征的眼部特征和先天性青光眼的模型的效用。使用光学相干断层扫描（OCT）和眼底照相术对Pitx 2 +/-和野生型同窝仔的眼睛进行临床检查。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PITX 2表达、结构完整性以及视神经和神经节细胞含量。PITX 2在出生后存在于角膜内皮和基质、虹膜基质、小梁网和施累姆氏管中。中央角膜厚度减少、虹膜缺损和虹膜粘连均在Pitx 2 +/−眼中普遍存在。虽然出生后第7天视神经头看起来正常，但到3周龄时，Pitx 2 +/−眼中IOP升高，视神经头杯状突起完全穿透。在3周龄时，Pitx 2 +/−小鼠的视神经中存在显著百分比的神经变性，并且在2月龄时完全渗透。Pitx 2 +/-眼在2月龄时在所有四个象限中显示出特异性神经节细胞密度的显著降低。Pitx 2 +/−小鼠模型的主要眼部特征的Axenel-Rieger综合征，将是一个重要的资源，了解分子机制，导致眼前段发育不全和青光眼的高患病率在这种疾病。此外，这些小鼠可能提供一种有效的新模型，用于更普遍地评估青光眼中的分子事件，并用于开发和测试这种疾病的新治疗模式。
The purpose of this analysis was to assess the utility of Pitx2+/− mice as a model for the ocular features of Axenfeld-Rieger Syndrome and for congenital glaucoma. Eyes of Pitx2+/− 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. 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+/− eyes. Although optic nerve heads appear normal at postnatal day 7, IOP is elevated and optic nerve head cupping is fully penetrant in Pitx2+/− eyes by 3 weeks of age. Neurodegeneration is present in a significant percentage of optic nerves from Pitx2+/− mice by 3 weeks of age, and is fully penetrant by 2 months of age. Pitx2+/− eyes show significant reductions in specifically ganglion cell density in all four quadrants by 2 months of age. Pitx2+/− 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.
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影响因子：
2.9
作者：
Savinova OV;Sugiyama F;Martin JE;Tomarev SI;Paigen BJ;Smith RS;John SW
通讯作者：
John SW
影响因子：
3.9
作者：
Kim, C. Y.;Kuehn, M. H.;Kwon, Y. H.
通讯作者：
Kwon, Y. H.
视网膜神经节细胞变性是拓扑的，但在DBA/2J小鼠中不是特异性细胞类型。
影响因子：
7.8
作者：
Jakobs, Tatjana C;Libby, Richard T;Ben, Yixin;John, Simon W M;Masland, Richard H
通讯作者：
Masland, Richard H
DBA/2J小鼠的进行性神经节细胞丧失和视神经变性是可变且不对称的。
影响因子：
2.4
作者：
Schlamp, Cassandra L.;Li, Yan;Dietz, Joel A.;Janssen, Katherine T.;Nickells, Robert W.
通讯作者：
Nickells, Robert W.
DOI：
10.1097/00005072-198303000-00009
发表时间：
1983-01-01
影响因子：
3.2
作者：
SADUN, AA;SMITH, LEH;KENYON, KR
通讯作者：
KENYON, KR