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UPL大鼠早发性遗传性白内障的晶状体发育及晶状体蛋白分布

Lens development and crystallin distribution of the early onset hereditary cataract in the UPL rat.

作者信息

Tomohiro M, Murata S, Yazawa K, Shinzawa S, Maruyama Y, Uga S, Mizuno A, Sakuma S

机构信息

Upjohn Pharmaceuticals Limited, Tsukuba Research Laboratories, Japan.

出版信息

Jpn J Ophthalmol. 1996;40(1):42-52.

PMID:8739499
Abstract

The Upjohn Pharmaceuticals Limited (UPL) rat is a new dominant hereditary cataract model with two types of cataract formation, early onset (E-type) and late onset (L-type). In the UPL E-type rat cataracts have formed by the time the eyes open. The E-type rat also develops microphthalmos and buphthalmos. In this study, histological observations of cataract development in the E-type rats were performed during the period of fetal development, at birth and at 1, 3 and 62 weeks of age. In addition, the distribution of alpha- and gamma-crystallins in the developing lens was determined using anti-crystallin antisera. Abnormal elongation of the lens fibers was first observed on the 13th day of gestation. Stratification of lens epithelial cells was present on the 19th day of gestation and this change became more severe with age. There was no alteration in alpha- and gamma-crystallin expression during the fetal period. The continuity between the lens epithelial cells and lens fibers was lost at 3 weeks of age and it was assumed that differentiation of lens epithelial cells had ceased. These findings suggested that development of the UPL rat E-type cataract was related to an early abnormality in lens cell differentiation not involving crystallin expression.

摘要

Upjohn制药有限公司(UPL)大鼠是一种新的显性遗传性白内障模型,有两种类型的白内障形成,即早发型(E型)和晚发型(L型)。在UPL E型大鼠中,眼睛睁开时白内障已经形成。E型大鼠还会出现小眼球和大眼球。在本研究中,对E型大鼠在胎儿发育期间、出生时以及1、3和62周龄时白内障的发展进行了组织学观察。此外,使用抗晶状体蛋白抗血清确定了发育中的晶状体中α-和γ-晶状体蛋白的分布。在妊娠第13天首次观察到晶状体纤维异常伸长。在妊娠第19天出现晶状体上皮细胞分层,且这种变化随年龄增长而更加严重。在胎儿期,α-和γ-晶状体蛋白表达没有改变。在3周龄时,晶状体上皮细胞与晶状体纤维之间的连续性丧失,推测晶状体上皮细胞的分化已经停止。这些发现表明,UPL大鼠E型白内障的发展与晶状体细胞分化的早期异常有关,而不涉及晶状体蛋白的表达。

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