THE RETINAL DEGENERATION SLOW (RDS) GENE-PRODUCT IS A PHOTORECEPTOR DISK MEMBRANE-ASSOCIATED GLYCOPROTEIN

THE RETINAL DEGENERATION SLOW (RDS) GENE-PRODUCT IS A PHOTORECEPTOR DISK MEMBRANE-ASSOCIATED GLYCOPROTEIN
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DOI:
10.1016/0896-6273(91)90122-g
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发表时间:
1991-01-01
期刊:
影响因子:
16.2
通讯作者:
BOK, D
BOK, D
中科院分区:
医学1区
文献类型:
--
作者:
TRAVIS, GH;SUTCLIFFE, JG;BOK, D

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视网膜变性慢速(rds)突变纯合子小鼠表现出感光细胞发育异常,随后出现缓慢变性。我们最近克隆了rds基因并确定了野生型rds mRNA的结构。在这里,我们证明该基因仅在感光细胞中表达。我们证明它编码一种 39 kd 的膜相关糖蛋白,该糖蛋白仅限于光感受器外节。通过电子显微镜,我们发现 rds 蛋白均匀分布在外节椎间盘内。 rds 蛋白的发育外观与外节椎间盘的形成一致。我们建议 rds 蛋白作为粘附分子来稳定外节椎间盘。
Mice homozygous for the retinal degeneration slow (rds) mutation exhibit abnormal development of photoreceptor cells, followed by their slow degeneration. We have recently cloned the rds gene and determined the structure of the wild-type rds mRNA. Here we show that the gene is expressed exclusively in photoreceptor cells. We demonstrate that it encodes a 39 kd membrane-associated glycoprotein that is restricted to photoreceptor outer segments. By electron microscopy, we show that the rds protein is distributed uniformly within outer segment discs. The developmental appearance of the rds protein coincides with outer segment disc formation. We propose that the rds protein functions as an adhesion molecule for stabilization of the outer segment discs.