视网膜色素变性：钙通道阻滞剂对rd小鼠视杆光感受器的挽救作用

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

作者信息

Frasson M, Sahel J A, Fabre M, Simonutti M, Dreyfus H, Picaud S

机构信息

Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, Médicale A, BP 426, 1 place de l'hôpital, Université Louis Pasteur, 67091 Strasbourg, France.

出版信息

Nat Med. 1999 Oct;5(10):1183-7. doi: 10.1038/13508.

DOI:10.1038/13508

PMID:10502823

Abstract

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.

摘要

视网膜色素变性是一种导致失明的遗传性光感受器退行性疾病。视网膜变性小鼠为这种疾病提供了一个特征明确的模型，在该模型中，视杆细胞环磷酸鸟苷磷酸二酯酶基因发生了突变，就像一些患病的人类家族一样。我们报告称，D-顺式地尔硫䓬，一种也作用于光敏感环磷酸鸟苷门控通道的钙通道阻滞剂，挽救了视网膜变性小鼠的光感受器并保留了视觉功能。地尔硫䓬在心脏病学领域长期的处方记录应有助于设计针对某些形式视网膜色素变性的临床试验。