CEP290 tethers flagellar transition zone microtubules to the membrane and regulates flagellar protein content.
CEP290 tethers flagellar transition zone microtubules to the membrane and regulates flagellar protein content.
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DOI：
10.1083/jcb.201006105
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发表时间：
2010-09-06
期刊：
影响因子：
--
通讯作者：
Witman GB
中科院分区：
文献类型：
--
作者：
Craige B;Tsao CC;Diener DR;Hou Y;Lechtreck KF;Rosenbaum JL;Witman GB
蛋白质进入和离开鞭毛是通过过渡区中的CEP 290来测量的。人CEP 290的突变导致纤毛相关疾病，其严重程度从孤立性失明到围产期致死。在这里，我们描述了一个衣原体reinhardtii突变体，其中大部分的CEP 290基因被删除。免疫电镜观察表明，CEP 290位于鞭毛过渡区，与那里突出的微管-膜连接密切相关。超微结构分析显示，在这些微管膜连接器的缺陷，导致鞭毛膜的连接损失的过渡区微管。分离的鞭毛的生化分析表明，突变体鞭毛有异常的蛋白质含量，包括鞭毛内转运蛋白和纤毛相关蛋白的异常水平。双核体实验表明，CEP 290在过渡区是动态的，经历快速的周转。结果表明，CEP 290是必需的，以形成微管膜连接器，拴系鞭毛膜的过渡区微管，是必不可少的控制鞭毛蛋白组成。
Entry and exit of proteins into flagella is gauged by CEP290 in the transition zone. Mutations in human CEP290 cause cilia-related disorders that range in severity from isolated blindness to perinatal lethality. Here, we describe a Chlamydomonas reinhardtii mutant in which most of the CEP290 gene is deleted. Immunoelectron microscopy indicated that CEP290 is located in the flagellar transition zone in close association with the prominent microtubule–membrane links there. Ultrastructural analysis revealed defects in these microtubule–membrane connectors, resulting in loss of attachment of the flagellar membrane to the transition zone microtubules. Biochemical analysis of isolated flagella revealed that the mutant flagella have abnormal protein content, including abnormal levels of intraflagellar transport proteins and proteins associated with ciliopathies. Experiments with dikaryons showed that CEP290 at the transition zone is dynamic and undergoes rapid turnover. The results indicate that CEP290 is required to form microtubule–membrane linkers that tether the flagellar membrane to the transition zone microtubules, and is essential for controlling flagellar protein composition.
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DOI：
10.1083/jcb.102.5.1797
发表时间：
1986-05
期刊：
The Journal of cell biology
影响因子：
--
作者：
Bloodgood RA;Woodward MP;Salomonsky NL
通讯作者：
Salomonsky NL
DOI：
10.1016/j.bbrc.2004.09.133
发表时间：
2004-11-12
影响因子：
3.1
作者：
Guo, JH;Jin, GL;Shou, CC
通讯作者：
Shou, CC
影响因子：
4
作者：
Hao, Limin;Scholey, Jonathan M.
通讯作者：
Scholey, Jonathan M.
影响因子：
4.2
作者：
Hauswirth, William W.;Aleman, Tomas S.;Jacobson, Samuel G.
通讯作者：
Jacobson, Samuel G.
DOI：
10.1083/jcb.141.4.993
发表时间：
1998-05-18
期刊：
The Journal of cell biology
影响因子：
--
作者：
Cole DG;Diener DR;Himelblau AL;Beech PL;Fuster JC;Rosenbaum JL
通讯作者：
Rosenbaum JL