ROD-CONE DYSPLASIA IN IRISH SETTERS - DEFECT IN CYCLIC-GMP METABOLISM IN VISUAL CELLS
ROD-CONE DYSPLASIA IN IRISH SETTERS - DEFECT IN CYCLIC-GMP METABOLISM IN VISUAL CELLS
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DOI：
10.1126/science.210508
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发表时间：
1978-01-01
期刊：
影响因子：
56.9
通讯作者：
CHADER, GJ
中科院分区：
文献类型：
--
作者：
AGUIRRE, G;FARBER, D;CHADER, GJ
爱尔兰赛特犬遗传性视杆-视锥发育不良表现为视网膜循环[c]GMP代谢异常。受影响的视觉细胞缺乏cGMP磷酸二酯酶活性，cGMP水平升高。在爱尔兰赛特犬的视网膜中观察到的生化异常与遗传性视网膜退化小鼠在视觉细胞退化开始之前的视网膜生化异常相似。CGMP代谢缺陷可能是早期视网膜退行性疾病的特征，可能包括那些影响人类的疾病。
An abnormality in retinal cyclic[c]GMP metabolism is demonstrated in the inherited rod-cone dysplasia of Irish Setter dogs. Affected visual cells are deficient in cGMP phosphodiesterase activity and have elevated levels of cGMP. The biochemical abnormalities observed in affected retinas of Irish Setters are similar to those in the retinas of mice with inherited retinal degeneration before visual cell degeneration begins. A defect in cGMP metabolism may be characteristic of early-onset degenerative diseases of the retina, possibly including those that affect humans.