THE BETA-SUBUNIT OF CYCLIC-GMP PHOSPHODIESTERASE MESSENGER-RNA IS DEFICIENT IN CANINE ROD CONE DYSPLASIA-1

被引:79
作者
FARBER, DB
DANCIGER, JS
AGUIRRE, G
机构
[1] UNIV CALIF LOS ANGELES,SCH MED,DEPT OPHTHALMOL,LOS ANGELES,CA 90024
[2] UNIV CALIF LOS ANGELES,SCH VET MED,MED GENET SECT,LOS ANGELES,CA 90024
[3] UNIV PENN,SCH MED,SCHEIE EYE INST,PHILADELPHIA,PA 19104
[4] UNIV PENN,SCH MED,DEPT OPHTHALMOL,PHILADELPHIA,PA 19104
关键词
D O I
10.1016/0896-6273(92)90173-B
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Irish setter dogs affected with rod-cone dysplasia 1 have elevated levels of retinal cGMP resulting from deficient rod-specific cGMP phosphodiesterase (cGMP PDE) activity. We investigated the mRNAs coding for the three subunits of cGMP PDE and for the proteins involved in the activation/deactivation of this enzyme in the retinas of developing affected and control dogs. While the photoreceptor cells are viable in the diseased retinas, opsin, transducin alpha-1 and beta-1, 48 and 33 kd proteins, and cGMP PDE-alpha and gamma-mRNAs have normal transcript sizes and levels. In contrast, a different pattern of cGMP PDE-beta mRNAs with lower than normal concentrations is present in the developing affected retinas prior to degeneration. Our observations suggest that an abnormality involving cGMP PDE-beta expression is implicated in rod-cone dysplasia 1.
引用
收藏
页码:349 / 356
页数:8
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