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Autophagy:自噬参与Vici综合征致病机理

2018-01-03 海北 MedSci原创

Vici综合征是由EPG5基因的隐性突变引起的人类遗传性多系统疾病,已有的研究显示,EPG5蛋白可以参与介导自噬体与溶酶体的融合。

Vici综合征是由EPG5基因的隐性突变引起的人类遗传性多系统疾病,已有的研究显示,EPG5蛋白可以参与介导自噬体与溶酶体的融合。该疾病的特征包括免疫缺陷,如记忆B细胞的缺乏,以及对感染的易感性的增加等。但是至今为止,EPG5免疫系统中的作用仍然是未知的。

近日,来自意大利IRCCS巴比诺GES U儿童医院的研究人员发现,EPG5对于TLR9配体CpG运输到晚期核内体溶酶体区室,以及TLR9信号传递的启动是必不可少的。TLR9信号传递的启动是人类记忆B细胞的生存,以及其最终分化为浆细胞必不可少的一步。

此外,EPG5的预测结构包括膜重塑结构域和核生成蛋白样结构域,这解释了其如何承担相互分开的小泡之间的载体的功能。

因此,研究人员的研究结果表明,EPG5通过控制核酸在细胞内的运输,连接了巨自噬/自噬与先天和适应性免疫。


原始出处:

E. Piano Mortari et al. The Vici syndrome protein EPG5 regulates intracellular nucleic acid trafficking linking autophagy to innate and adaptive immunityAutophagy, 2017; DOI: https://doi.org/10.1080/15548627.2017.1389356


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    2018-01-04 飘飘爱

    很好

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=2010669, encodeId=b13a2010669b0, content=<a href='/topic/show?id=ce9086483c8' target=_blank style='color:#2F92EE;'>#致病机理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=86483, encryptionId=ce9086483c8, topicName=致病机理)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=649f191, createdName=zzc2211, createdTime=Mon Feb 05 02:44:00 CST 2018, time=2018-02-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2086211, encodeId=5aec208621134, content=<a href='/topic/show?id=74b118425af' target=_blank style='color:#2F92EE;'>#Vici综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=56, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=18425, encryptionId=74b118425af, topicName=Vici综合征)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6cc1239, createdName=sodoo, createdTime=Thu Mar 08 11:44:00 CST 2018, time=2018-03-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431061, encodeId=c9d2143106102, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 04:44:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275316, encodeId=f01d2e5316ad, content=很好, beContent=null, objectType=article, channel=null, level=null, likeNumber=85, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib2emBzjO0FtqTJ05AqrEEdRmp6tmlsLXqpzfxWdtgbbgPGWRHagoK9fLTd3IayRPgkJVIVBWtdlmnkBhOIR4cFM/0, createdBy=e8d51649330, createdName=飘飘爱, createdTime=Thu Jan 04 08:42:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275300, encodeId=7fb92e5300f0, content=学习了.涨知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=84, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20220115/46bcf39c32de4aa6b45c5f9d66c8ee77/6cb4a20c55bb4b7691122f47747bfca2.jpg, createdBy=9dad1662329, createdName=1ddf0692m34(暂无匿称), createdTime=Thu Jan 04 07:46:52 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275172, encodeId=12d72e517203, content=了解了解.继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=82, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=93f31631454, createdName=1dd8c52fm63(暂无匿称), createdTime=Wed Jan 03 23:15:11 CST 2018, time=2018-01-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275068, encodeId=5f6f2e5068b1, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=101, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4NfDEeydJz0GlP8O0ngrUX6AYCXFic0XDOOAyeQicIqnOd4TrWSwiagJrsFNYuWC1Uc6iavbS1hWt9xtZAhAqLGHqZyl/0, createdBy=42891977006, createdName=1e17414fm02(暂无匿称), createdTime=Wed Jan 03 14:17:29 CST 2018, time=2018-01-03, status=1, ipAttribution=)]
    2018-01-04 1ddf0692m34(暂无匿称)

    学习了.涨知识

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=2010669, encodeId=b13a2010669b0, content=<a href='/topic/show?id=ce9086483c8' target=_blank style='color:#2F92EE;'>#致病机理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=86483, encryptionId=ce9086483c8, topicName=致病机理)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=649f191, createdName=zzc2211, createdTime=Mon Feb 05 02:44:00 CST 2018, time=2018-02-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2086211, encodeId=5aec208621134, content=<a href='/topic/show?id=74b118425af' target=_blank style='color:#2F92EE;'>#Vici综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=56, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=18425, encryptionId=74b118425af, topicName=Vici综合征)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6cc1239, createdName=sodoo, createdTime=Thu Mar 08 11:44:00 CST 2018, time=2018-03-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431061, encodeId=c9d2143106102, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 04:44:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275316, encodeId=f01d2e5316ad, content=很好, beContent=null, objectType=article, channel=null, level=null, likeNumber=85, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib2emBzjO0FtqTJ05AqrEEdRmp6tmlsLXqpzfxWdtgbbgPGWRHagoK9fLTd3IayRPgkJVIVBWtdlmnkBhOIR4cFM/0, createdBy=e8d51649330, createdName=飘飘爱, createdTime=Thu Jan 04 08:42:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275300, encodeId=7fb92e5300f0, content=学习了.涨知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=84, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20220115/46bcf39c32de4aa6b45c5f9d66c8ee77/6cb4a20c55bb4b7691122f47747bfca2.jpg, createdBy=9dad1662329, createdName=1ddf0692m34(暂无匿称), createdTime=Thu Jan 04 07:46:52 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275172, encodeId=12d72e517203, content=了解了解.继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=82, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=93f31631454, createdName=1dd8c52fm63(暂无匿称), createdTime=Wed Jan 03 23:15:11 CST 2018, time=2018-01-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275068, encodeId=5f6f2e5068b1, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=101, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4NfDEeydJz0GlP8O0ngrUX6AYCXFic0XDOOAyeQicIqnOd4TrWSwiagJrsFNYuWC1Uc6iavbS1hWt9xtZAhAqLGHqZyl/0, createdBy=42891977006, createdName=1e17414fm02(暂无匿称), createdTime=Wed Jan 03 14:17:29 CST 2018, time=2018-01-03, status=1, ipAttribution=)]
    2018-01-03 1dd8c52fm63(暂无匿称)

    了解了解.继续关注

    0

  7. [GetPortalCommentsPageByObjectIdResponse(id=2010669, encodeId=b13a2010669b0, content=<a href='/topic/show?id=ce9086483c8' target=_blank style='color:#2F92EE;'>#致病机理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=86483, encryptionId=ce9086483c8, topicName=致病机理)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=649f191, createdName=zzc2211, createdTime=Mon Feb 05 02:44:00 CST 2018, time=2018-02-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2086211, encodeId=5aec208621134, content=<a href='/topic/show?id=74b118425af' target=_blank style='color:#2F92EE;'>#Vici综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=56, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=18425, encryptionId=74b118425af, topicName=Vici综合征)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6cc1239, createdName=sodoo, createdTime=Thu Mar 08 11:44:00 CST 2018, time=2018-03-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431061, encodeId=c9d2143106102, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 04:44:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275316, encodeId=f01d2e5316ad, content=很好, beContent=null, objectType=article, channel=null, level=null, likeNumber=85, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib2emBzjO0FtqTJ05AqrEEdRmp6tmlsLXqpzfxWdtgbbgPGWRHagoK9fLTd3IayRPgkJVIVBWtdlmnkBhOIR4cFM/0, createdBy=e8d51649330, createdName=飘飘爱, createdTime=Thu Jan 04 08:42:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275300, encodeId=7fb92e5300f0, content=学习了.涨知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=84, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20220115/46bcf39c32de4aa6b45c5f9d66c8ee77/6cb4a20c55bb4b7691122f47747bfca2.jpg, createdBy=9dad1662329, createdName=1ddf0692m34(暂无匿称), createdTime=Thu Jan 04 07:46:52 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275172, encodeId=12d72e517203, content=了解了解.继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=82, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=93f31631454, createdName=1dd8c52fm63(暂无匿称), createdTime=Wed Jan 03 23:15:11 CST 2018, time=2018-01-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275068, encodeId=5f6f2e5068b1, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=101, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4NfDEeydJz0GlP8O0ngrUX6AYCXFic0XDOOAyeQicIqnOd4TrWSwiagJrsFNYuWC1Uc6iavbS1hWt9xtZAhAqLGHqZyl/0, createdBy=42891977006, createdName=1e17414fm02(暂无匿称), createdTime=Wed Jan 03 14:17:29 CST 2018, time=2018-01-03, status=1, ipAttribution=)]
    2018-01-03 1e17414fm02(暂无匿称)

    学习

    0

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