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视锥细胞环核苷酸门控通道研究进展
徐建华
张兆奉 [综述]
杜晶 [综述]
作者及单位信息
·
DOI： 10.3760/cma.j.issn.2095-0160.2015.06.020
Cyclic nucleotide-gated channels and retinal cone photoreceptor cells function
Xu Jianhua
Zhang Zhaofeng
Du Jing
Authors Info & Affiliations
Xu Jianhua
Shanghai Institute of Planned Parenthood Research, NPFPC Laboratory of Contraception and Devices, Shanghai 200032, China
Zhang Zhaofeng
Du Jing
·
DOI： 10.3760/cma.j.issn.2095-0160.2015.06.020
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摘要

环核苷酸门控（CNG）通道是由环核苷酸活化的离子通道，其在视觉和嗅觉信号传导过程中发挥重要作用。CNG通道蛋白由6个不同基因编码，包含4个A亚单元（A1～A4）和2个B亚单元（B1和B3），其中 CNGA3和 CNGB3基因突变与全色盲发病相关。近年来，大量研究表明对全色盲动物模型进行基因治疗后视网膜功能恢复效果明显。就视锥细胞CNG通道的功能研究和CNG通道缺陷小鼠模型的发病机制和基因治疗研究进行综述。

环核苷酸门控通道;视锥细胞;基因敲除
ABSTRACT

Cyclic nucleotide-gated (CNG) channels are ion channels which are activated by the binding of cyclic guanosine monophosphate (cGMP) or cyclic adenosine monophosphate (cAMP), they play a central role in the signal transduction pathways of vision and olfaction.Six different genes encode CNG protein, containing four A subunits (A1-A4) and two B subunits (B1 and B3). CNGA3 and CNGB3 have been found to be implicated in achromatopsia-associated mutations.Recently, a huge amount of researches showed the good responses to gene therapy in achromatopsia animal models.This article briefly reviewed the physiological roles of CNG channel in retinal cone photoreceptor cells and the recent research achievements of gene therapy in CNG channel-deficient mouse models with achromatopsia.

Cyclic nucleotide-gated channel;Retinal cone photoreceptor cells;Gene, knockout
Xu Jianhua, Email: mocdef.6ab21027uxj
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引用本文

徐建华,张兆奉,杜晶. 视锥细胞环核苷酸门控通道研究进展[J]. 中华实验眼科杂志,2015,33(6)：573-576.

DOI:10.3760/cma.j.issn.2095-0160.2015.06.020

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1985年，Fesenko等 [ 1 ]首次报道环磷酸鸟苷（cyclic guanosine monophosphate，cGMP）能直接活化视网膜视杆体中光依赖性通道，随后，在视锥体光感受器、嗅觉神经元和化学敏感性纤毛等组织中均发现了相似的通道，环核苷酸门控（cyclic nucleotide-gated，CNG）通道的研究快速发展。近年国际上有关CNG离子通道的研究报道大量涌现，已成为生命科学中的一个热点研究领域。就视锥细胞的CNG通道功能和CNG通道缺陷小鼠模型的发病机制和基因治疗研究进行综述。
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备注信息
A
徐建华，Email： mocdef.6ab21027uxj
B
国家自然科学基金项目 (81300556)
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