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环鸟苷酸积累导致光感受器细胞退化:一种遗传性疾病的模拟

Cyclic GMP accumulation causes degeneration of photoreceptor cells: simulation of an inherited disease.

作者信息

Lolley R N, Farber D B, Rayborn M E, Hollyfield J G

出版信息

Science. 1977 May 6;196(4290):664-6. doi: 10.1126/science.193183.

DOI:10.1126/science.193183
PMID:193183
Abstract

Guanosine 3',5'-monophosphate (cyclic GMP) metabolism in developing eye rudiments of Xenopus laevis embryos in culture is disrupted by the phosphodiesterase inhibitor isobutylmethylxanthine. At low concentrations of inhibitor the rudiments develop normally, but at higher concentrations of the inhibitor, cyclic GMP accumulates in the rudiments and the retinal photoreceptor cells degenerate selectively. The isobutylmethylxanthine-induced photoreceptor degeneration is associated with an accumulation of cyclic GMP and, in this respect, it stimulates an early biochemical defect in the inherited degenerative disease of rd mice.

摘要

在培养的非洲爪蟾胚胎发育中的眼原基中,磷酸二酯酶抑制剂异丁基甲基黄嘌呤会破坏鸟苷 3',5'-单磷酸(环鸟苷酸)的代谢。在低浓度抑制剂作用下,眼原基正常发育,但在高浓度抑制剂作用下,环鸟苷酸在眼原基中积累,视网膜光感受器细胞选择性退化。异丁基甲基黄嘌呤诱导的光感受器退化与环鸟苷酸的积累有关,在这方面,它引发了 rd 小鼠遗传性退行性疾病中的早期生化缺陷。

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Cyclic GMP accumulation causes degeneration of photoreceptor cells: simulation of an inherited disease.环鸟苷酸积累导致光感受器细胞退化:一种遗传性疾病的模拟
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Science. 1974 Nov 1;186(4162):449-51. doi: 10.1126/science.186.4162.449.

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