ROD-CONE DYSPLASIA IN IRISH SETTERS - DEFECT IN CYCLIC-GMP METABOLISM IN VISUAL CELLS

被引:157
作者
AGUIRRE, G
FARBER, D
LOLLEY, R
FLETCHER, RT
CHADER, GJ
机构
[1] VET ADM HOSP,SEPULVEDA,CA 91343
[2] UNIV CALIF LOS ANGELES,SCH MED,JULES STEIN EYE INST,LOS ANGELES,CA 90024
[3] NEI,BETHESDA,MD 20014
关键词
D O I
10.1126/science.210508
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
An abnormality in retinal guanosine 3′,5′-monophosphate (cyclic GMP) metabolism is demonstrated in the inherited rod-cone dysplasia of Irish Setter dogs. Affected visual cells are deficient in cyclic GMP phosphodiesterase activity and have elevated levels of cyclic GMP. 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 cyclic GMP metabolism may be characteristic of early-onset degenerative diseases of the retina, possibly including those that affect humans. Copyright © 1978 AAAS.
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页码:1133 / 1134
页数:2
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