Retinitis pigmentosa:: rod photoreceptor rescue by a calcium-channel blocker in the rd mouse

被引:188
作者
Frasson, M
Sahel, JA
Fabre, M
Simonutti, M
Dreyfus, H
Picaud, S
机构
[1] Univ Strasbourg 1, Lab Physiopathol Retinienne, F-67091 Strasbourg, France
[2] Univ Strasbourg 1, Lab Physiopathol Cellulaire & Mol Retine, F-67091 Strasbourg, France
[3] Univ Strasbourg 1, Inst Histol, F-67091 Strasbourg, France
关键词
D O I
10.1038/13508
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Retinitis pigmentosa is an inherited degenerative disease of photoreceptors leading to blindness. A well-characterized model for this disease is provided by the retinal degeneration mouse, in which the gene for the rod cGMP phosphodiesterase is mutated, as in some affected human families. We report that D-cis-diltiazem, a calcium-channel blocker that also acts at light-sensitive cGMP-gated channels, rescued photoreceptors and preserved visual function in the retinal degeneration mouse. The long record of diltiazem prescription in cardiology should facilitate the design of clinical trials for some forms of retinitis pigmentosa.
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页码:1183 / 1187
页数:5
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