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J Dent Res：纤毛病理学相关蛋白Tmem107在颅面发育过程中的功能研究

2017-10-17 lishiting MedSci原创

颅面发育纤毛蛋白功能
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一类纤毛病理学相关的人类广谱疾病是由于初级纤毛形态或信号转导发生缺陷所致。初级纤毛是一个单独的细胞器，它可以对来自于胞外和胞内的机械和化学刺激产生反应。跨膜蛋白107 (TMEM107)存在于初级纤毛上，并且富含于过渡带区域，可以调节纤毛的蛋白质含量。TMEM107缺陷最初与口-面-指综合征、美-格二氏综合征和Joubert综合征相关联，表现为一系列纤毛病理学相关的缺陷。在此，研究者通过在小鼠胚胎

一类纤毛病理学相关的人类广谱疾病是由于初级纤毛形态或信号转导发生缺陷所致。初级纤毛是一个单独的细胞器，它可以对来自于胞外和胞内的机械和化学刺激产生反应。跨膜蛋白107 (TMEM107)存在于初级纤毛上，并且富含于过渡带区域，可以调节纤毛的蛋白质含量。TMEM107缺陷最初与口-面-指综合征、美-格二氏综合征和Joubert综合征相关联，表现为一系列纤毛病理学相关的缺陷。在此，研究者通过在小鼠胚胎中完全敲除Tmem107基因，分析Tmem107在颅面发育中特别是上腭形成过程中发挥的作用。结果显示，Tmem107-/-小鼠表现为广谱的颅面发育缺损，包括：口鼻部短小、面中线延长、唇裂、大范围的露脑以及小眼畸形或无眼畸形。缺陷的外部异常情况发生在骨骼结构中，包括：在几个膜成骨中的骨化延迟和鼻中隔的扩展或犁鼻软骨缺陷。腭突的改变导致继发腭裂缝的形成。而腭缺损是由于间充质过度增殖导致腭突早期的过度发育，进而引起其水平化缺陷。另外，Tmem107-/-小鼠腭突的上皮干性标志物SOX2的表达改变以及间充质SOX9的表达差异表明神经嵴的迁移增强。对初级纤毛的详细分析显示，微管蛋白乙酰化和IFT88表达

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    2018-02-07 meichuangyi

    #TME#

    0

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    2017-11-08 wshxjq

    #纤毛病#

    0

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    2017-10-19 neurowu

    #发育#

    0

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    2017-10-19 jktdtl

    #病理学#

    0

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    2017-10-18 tanxingdoctor

    学习啦!谢谢分享

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1927507, encodeId=2568192e50797, content=<a href='/topic/show?id=b4931e55602' target=_blank style='color:#2F92EE;'>#TME#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=86, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=17556, encryptionId=b4931e55602, topicName=TME)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=08e4137, createdName=meichuangyi, createdTime=Wed Feb 07 11:36:00 CST 2018, time=2018-02-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1804446, encodeId=2637180444686, content=<a href='/topic/show?id=b34ce7210b3' target=_blank style='color:#2F92EE;'>#纤毛病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77210, encryptionId=b34ce7210b3, topicName=纤毛病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=fd34186, createdName=wshxjq, createdTime=Wed Nov 08 10:36:00 CST 2017, time=2017-11-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1347230, encodeId=1918134e2305d, content=<a href='/topic/show?id=88713e938c1' target=_blank style='color:#2F92EE;'>#发育#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=101, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37938, encryptionId=88713e938c1, topicName=发育)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f4c124, createdName=neurowu, createdTime=Thu Oct 19 14:36:00 CST 2017, time=2017-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1473447, encodeId=da8b14e344749, content=<a href='/topic/show?id=4fd1e102257' target=_blank style='color:#2F92EE;'>#病理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=77, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71022, encryptionId=4fd1e102257, topicName=病理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c8ce7226273, createdName=jktdtl, createdTime=Thu Oct 19 14:36:00 CST 2017, time=2017-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=253991, encodeId=4f0925399176, content=学习啦!谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=96, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLK1wzJZRTON4gxYUibhUyOypBwMibC4R7SsppicGhz5Ogoy0VVq4CWnqaD2Moeib8RWxI5lOiaVmnxpan/0, createdBy=c53f1730331, createdName=tanxingdoctor, createdTime=Wed Oct 18 09:50:41 CST 2017, time=2017-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=253981, encodeId=fa6625398167, content=学习了很有用, beContent=null, objectType=article, channel=null, level=null, likeNumber=135, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a6831717982, createdName=微分, createdTime=Wed Oct 18 09:25:23 CST 2017, time=2017-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=253893, encodeId=b25925389385, content=好好好好好好好好, beContent=null, objectType=article, channel=null, level=null, likeNumber=83, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/08/29/bbc5cc0eed9b8d17623d2fd49dbdf19a.jpg, createdBy=79522135794, createdName=yjs木玉, createdTime=Wed Oct 18 05:11:53 CST 2017, time=2017-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=253882, encodeId=f4d725388207, content=学习了很有用, beContent=null, objectType=article, channel=null, level=null, likeNumber=117, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib3IzoRn621JdKCFUI4XvNELcibocrAVU6aUzEVDSaf3rYH08PUYUiagGqxAEhj8g9WqqNvD1CupIpCrmnR2OrqHZh/0, createdBy=7f181365728, createdName=飛歌, createdTime=Wed Oct 18 02:55:12 CST 2017, time=2017-10-18, status=1, ipAttribution=)]
    2017-10-18 微分

    学习了很有用

    0

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    2017-10-18 yjs木玉

    好好好好好好好好

    0

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    2017-10-18 飛歌

    学习了很有用

    0