Asakawa M, Azuma N
Department of Ophthalmology, National Children's Hospital, Tokyo, Japan.
Nippon Ganka Gakkai Zasshi. 1997 Jan;101(1):87-91.
Distribution of glycosaminoglycans in the trabecular tissue was immunohistochemically investigated in 7 congenital aniridia eyes of 5 patients aged 0 to 43 days. Paraffin sections of each specimen were immunohistochemically stained with antibodies to chondoroitin (clone 1-B-5), chondoroitin-1-sulfate (2-B-6), chondoroitin-6-sulfate (3-B-3), dermatan sulfate (6-B-6), and keratan sulfate (5-D-4). The trabecular meshwork and Schlemm's canal in all eyes were absent or not well differentiated. The cornea, trabecular tissue, and iris stroma were negative for chondoroitin immunostaining but positive for chondoroitin-4-sulfate, chondoroitin-6-sulfate, dermatan sulfate and keratan sulfate immunostaining. In the normal anterior segment tissue keratan sulfate is present in the cornea, trabecular tissue, and iris at the fetal stage, and disappears from the iris at the neonatal stage. These findings suggest that the persistence and/or abnormal distribution of keratan sulfate in the anterior segment may play a role in the pathogenesis of congenital aniridia.
对5例年龄在0至43天的患者的7只先天性无虹膜眼小梁组织中的糖胺聚糖分布进行了免疫组织化学研究。对每个标本的石蜡切片用抗软骨素(克隆1-B-5)、硫酸软骨素-1(2-B-6)、硫酸软骨素-6(3-B-3)、硫酸皮肤素(6-B-6)和硫酸角质素(5-D-4)的抗体进行免疫组织化学染色。所有眼睛的小梁网和施莱姆管均缺失或分化不良。角膜、小梁组织和虹膜基质对软骨素免疫染色呈阴性,但对硫酸软骨素-4、硫酸软骨素-6、硫酸皮肤素和硫酸角质素免疫染色呈阳性。在正常眼前段组织中,硫酸角质素在胎儿期存在于角膜、小梁组织和虹膜中,在新生儿期从虹膜中消失。这些发现表明,硫酸角质素在前房的持续存在和/或异常分布可能在先天性无虹膜的发病机制中起作用。