Characterization of the medaka(Oryzias latipes) primary ciliary dyskinesia mutant, jaodori.

青鳉（Oryzias latipes）原发性纤毛运动障碍突变体 jaodori 的表征。

• 批准号: 21590197
• 负责人: KOBAYASHI Daisuke
• 金额: $ 2.91万
• 依托单位: Kyoto Prefectural University of Medicine
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2009
• 资助国家: 日本
• 起止时间: 2009 至 2011
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21590197/
• 关键词: 発生・文化; 運動性繊毛; ダイニン; 発生; 発生・分化; 運動性纎毛

Cilia and flagella are highly conserved organelles that have diverse motility and sensory functions. Motility defects in cilia and flagella result in primary ciliary dyskinesia(PCD). We isolated a novel medaka PCD mutant, jaodori(joi). Positional cloning showed that axonemal dynein intermediate chain 2(dnai2) is responsible for joi. In the joi mutant, cilia in Kupffer's vesicle(KV), an organ functionally equivalent to the mouse node in terms of left-right specification, are generated but their motility is disrupted, resulting in a LR defect. Ultrastructural analysis revealed severe reduction in the outer dynein arms in KV cilia of joi mutants.

纤毛和鞭毛是高度保守的细胞器，具有多种运动和感觉功能。纤毛和鞭毛的运动缺陷导致原发性纤毛运动障碍（PCD）。我们分离出一种新的青鳉 PCD 突变体 jaodori(joi)。定位克隆表明轴丝动力蛋白中间链2（dnai2）负责joi。在joi突变体中，库普弗囊泡（KV）中产生了纤毛，该器官在功能上与小鼠节点在左右规范方面相当，但它们的运动性被破坏，导致LR缺陷。超微结构分析显示 joi 突变体 KV 纤毛的外动力蛋白臂严重减少。

项目成果

Medaka : Biology, Management, and Experimental Protocols

青鳉：生物学、管理和实验方案

• 发表时间: 2009
• 作者: Editors : Masato Kinoshita; Kenji Murata; Kiyoshi Naruse; Minoru Tanaka(分担執筆者:小林大介)
• 通讯作者: Minoru Tanaka(分担執筆者:小林大介)

An attempt to identify novel ciliary genes by database comparison

通过数据库比较鉴定新的睫状体基因的尝试

• 发表时间: 2012
• 作者: Daisuke Kobayashi; Dai Shiba; Takahiko Yokoyama
• 通讯作者: Takahiko Yokoyama

haracterization of the medaka(Oryzias latipes) primary ciliary dyskinesia mutant, jaodori : Redundant and distinct roles of two dynein axonemal intermediate chain 2s(dnai2s) in motile cilia

青鳉（Oryzias latipes）原发性纤毛运动障碍突变体 jaodori 的特征：运动纤毛中两条动力蛋白轴丝中间链 2s（dnai2s）的冗余和独特作用

• 作者: 小林大介;飯島典生;萩原治夫;加村敬一郎;武田洋幸;横山尚彦
• 通讯作者: 横山尚彦

Characterization of the medaka (Oryzias latipes) primary ciliary dyskinesia mutant, jaodori : Redundant and distinct roles of two dynein axonemal intermediate chain 2s (dnai2s) in motile cilia

青鳉 (Oryzias latipes) 原发性纤毛运动障碍突变体 jaodori 的表征：运动纤毛中两条动力蛋白轴丝中间链 2s (dnai2s) 的冗余和独特作用

• 发表时间: 2011
• 作者: Daisuke Kobayashi; Norio Iijima; Haruo Hagiwara;; Keiichiro Kamura; Hiroyuki Takeda; Takahiko Yokoyama
• 通讯作者: Takahiko Yokoyama

Medaka : Model for Organogenesis, Human Disease, and Evolution

青鳉：器官发生、人类疾病和进化模型

• 发表时间: 2011
• 作者: Kobayashi D.; Takeda H. Takeda H; Naruse K; Tanaka M
• 通讯作者: Tanaka M