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[实验性小眼畸形小鼠布鲁赫膜中糖胺聚糖的组织化学研究]

[Histochemical studies on glycosaminoglycans in Bruch's membrane of experimental microphthalmic mice].

作者信息

Uno M, Ikeda K, Majima A, Hirabayashi Y, Yamada K

机构信息

Department of Ophthalmology, Nagoya City University Medical School.

出版信息

Nippon Ganka Gakkai Zasshi. 1996 Feb;100(2):126-31.

PMID:8851151
Abstract

The Cts mouse is a mutant mouse of the Jcl:ICR strain which has congenital cataracts and small eyes in homozygotes. Therefore, this mouse could be a model for autosomal dominant cataract with microphthalmia in humans. In the present study, attempts were made to demonstrate light-microscopically the molecular species of glycosaminoglycans (GAG) in Bruch's membrane of Cts mice on day 14, 16, and 18 of gestation and at 0, 4, and 7 days after birth by means of combined sensitized high iron diamine staining and testicular hyarulonidase digestion procedures. In Bruch's membrane of the unaffected mice at all stages examined, no substantial amount of chondroitin sulfate A/C could be detected. In homozygous mice, however, a large amount of chondroitin sulfate A/C was contained in Bruch's membrane. These results indicate that the abnormal change in GAG molecular species induced the functional disorder of Bruch's membrane and could play a certain role in the etiology of microphthalmia in homozygotes.

摘要

Cts小鼠是Jcl:ICR品系的突变小鼠,纯合子具有先天性白内障和小眼睛。因此,这种小鼠可能是人类常染色体显性白内障伴小眼球症的模型。在本研究中,尝试通过联合致敏高铁二胺染色和睾丸透明质酸酶消化程序,在妊娠第14、16和18天以及出生后0、4和7天,用光镜观察Cts小鼠布鲁赫膜中糖胺聚糖(GAG)的分子种类。在所检查的所有阶段的未受影响小鼠的布鲁赫膜中,未检测到大量硫酸软骨素A/C。然而,在纯合子小鼠中,布鲁赫膜中含有大量硫酸软骨素A/C。这些结果表明,GAG分子种类的异常变化导致了布鲁赫膜的功能障碍,并且可能在纯合子小眼球症的病因中起一定作用。

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