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Mov Disord:遗传性痉挛性截瘫亚型脑损伤的病理生理机制不同

2021-06-28 MedSci原创 MedSci原创

遗传性痉挛性截瘫
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脑损伤是HSPs的一个显著特征（即使是单纯亚型），但其异常模式具有基因型特异性。

脊髓一直被认为是遗传性痉挛性截瘫（HSPs）的主要损伤靶点，但越来越多的证据表明大脑也受到影响。尽管如此，人们对热休克蛋白的脑信号知之甚少，特别是对特定基因亚型的分层。 Katiane R Servelhere等研究了5种热休克蛋白亚型（9spg3a、27spg4、10spg7、9spg8和29spg11）的脑和小脑损伤特点，揭示其临床和基因表达的相关性。研究结果发表在Movement Disorders杂志。 在该横断面病例对照研究（n=84）中获得高分辨率脑T1和弥散张量图像（DTI）数据集。MRICloud、FreeSurfer和CERES-SUIT方法被用来评估大脑灰质（GM）和白质（WM）以及小脑。 研究方案 （上）与年龄和性别匹配的健康对照相比，SPG4患者的脑结构损害。灰质损害与皮质厚度和深核体积测量结果相对应。白质损害与平均扩散系数的发现相对应。（中）脑SPAST基因表达覆盖在FreeSurfer标签上。颜色条代表显著的错误发现率(FDR)校正的P值和Z-分数中的基因表达。（底部）结构损伤图（以蓝色显示）和SPAST表达式（以红色显示）的叠加图。损伤和表达重叠的区域以粉

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    2021-10-22 般若傻瓜

    #遗传性#

    0

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    2022-02-21 许安

    #病理生理#

    0

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    2022-02-14 cmsvly

    #Disord#

    0

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    2021-11-12 cmj8wellington

    #Dis#

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=1860148, encodeId=5841186014891, content=<a href='/topic/show?id=cc73951054b' target=_blank style='color:#2F92EE;'>#遗传性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=95105, encryptionId=cc73951054b, topicName=遗传性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ad9f79, createdName=般若傻瓜, createdTime=Fri Oct 22 23:53:27 CST 2021, time=2021-10-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701789, encodeId=ee7d1e0178947, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Mon Feb 21 16:53:27 CST 2022, time=2022-02-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784436, encodeId=6d291e8443605, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=33, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Mon Feb 14 17:53:27 CST 2022, time=2022-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013648, encodeId=28472013648d7, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Fri Nov 12 04:53:27 CST 2021, time=2021-11-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1311792, encodeId=d79f1311e92d3, content=<a href='/topic/show?id=624de11204a' target=_blank style='color:#2F92EE;'>#痉挛#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71120, encryptionId=624de11204a, topicName=痉挛)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7dc7399, createdName=apoenzyme, createdTime=Wed Jun 30 07:53:27 CST 2021, time=2021-06-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1398155, encodeId=d41b139815515, content=<a href='/topic/show?id=8bee565436d' target=_blank style='color:#2F92EE;'>#损伤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=56543, encryptionId=8bee565436d, topicName=损伤)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/DYAIOgq83eoNX3lUOlDKGBUEPNwZx0nibmdcFyeibnX4WMhUSCU2REpGRWtSmnT2CCs0dA5waxvJknYDxwtYnIcQ/132, createdBy=294e2500218, createdName=ms3046638856685384, createdTime=Wed Jun 30 07:53:27 CST 2021, time=2021-06-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1039429, encodeId=bead10394292e, content=机制研究离临床仍然有距离，不过与临床结合思考，仍然有帮助的，不能仅仅是纯临床思维，转化思维同样重要, beContent=null, objectType=article, channel=null, level=null, likeNumber=50, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=肿肿, createdTime=Mon Jun 28 19:53:27 CST 2021, time=2021-06-28, status=1, ipAttribution=)]
    2021-06-30 apoenzyme

    #痉挛#

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1860148, encodeId=5841186014891, content=<a href='/topic/show?id=cc73951054b' target=_blank style='color:#2F92EE;'>#遗传性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=95105, encryptionId=cc73951054b, topicName=遗传性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ad9f79, createdName=般若傻瓜, createdTime=Fri Oct 22 23:53:27 CST 2021, time=2021-10-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701789, encodeId=ee7d1e0178947, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Mon Feb 21 16:53:27 CST 2022, time=2022-02-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784436, encodeId=6d291e8443605, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=33, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Mon Feb 14 17:53:27 CST 2022, time=2022-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013648, encodeId=28472013648d7, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Fri Nov 12 04:53:27 CST 2021, time=2021-11-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1311792, encodeId=d79f1311e92d3, content=<a href='/topic/show?id=624de11204a' target=_blank style='color:#2F92EE;'>#痉挛#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71120, encryptionId=624de11204a, topicName=痉挛)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7dc7399, createdName=apoenzyme, createdTime=Wed Jun 30 07:53:27 CST 2021, time=2021-06-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1398155, encodeId=d41b139815515, content=<a href='/topic/show?id=8bee565436d' target=_blank style='color:#2F92EE;'>#损伤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=56543, encryptionId=8bee565436d, topicName=损伤)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/DYAIOgq83eoNX3lUOlDKGBUEPNwZx0nibmdcFyeibnX4WMhUSCU2REpGRWtSmnT2CCs0dA5waxvJknYDxwtYnIcQ/132, createdBy=294e2500218, createdName=ms3046638856685384, createdTime=Wed Jun 30 07:53:27 CST 2021, time=2021-06-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1039429, encodeId=bead10394292e, content=机制研究离临床仍然有距离，不过与临床结合思考，仍然有帮助的，不能仅仅是纯临床思维，转化思维同样重要, beContent=null, objectType=article, channel=null, level=null, likeNumber=50, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=肿肿, createdTime=Mon Jun 28 19:53:27 CST 2021, time=2021-06-28, status=1, ipAttribution=)]
    2021-06-30 ms3046638856685384

    #损伤#

    0

  7. [GetPortalCommentsPageByObjectIdResponse(id=1860148, encodeId=5841186014891, content=<a href='/topic/show?id=cc73951054b' target=_blank style='color:#2F92EE;'>#遗传性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=95105, encryptionId=cc73951054b, topicName=遗传性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ad9f79, createdName=般若傻瓜, createdTime=Fri Oct 22 23:53:27 CST 2021, time=2021-10-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701789, encodeId=ee7d1e0178947, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Mon Feb 21 16:53:27 CST 2022, time=2022-02-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784436, encodeId=6d291e8443605, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=33, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Mon Feb 14 17:53:27 CST 2022, time=2022-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013648, encodeId=28472013648d7, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Fri Nov 12 04:53:27 CST 2021, time=2021-11-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1311792, encodeId=d79f1311e92d3, content=<a href='/topic/show?id=624de11204a' target=_blank style='color:#2F92EE;'>#痉挛#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71120, encryptionId=624de11204a, topicName=痉挛)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7dc7399, createdName=apoenzyme, createdTime=Wed Jun 30 07:53:27 CST 2021, time=2021-06-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1398155, encodeId=d41b139815515, content=<a href='/topic/show?id=8bee565436d' target=_blank style='color:#2F92EE;'>#损伤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=56543, encryptionId=8bee565436d, topicName=损伤)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/DYAIOgq83eoNX3lUOlDKGBUEPNwZx0nibmdcFyeibnX4WMhUSCU2REpGRWtSmnT2CCs0dA5waxvJknYDxwtYnIcQ/132, createdBy=294e2500218, createdName=ms3046638856685384, createdTime=Wed Jun 30 07:53:27 CST 2021, time=2021-06-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1039429, encodeId=bead10394292e, content=机制研究离临床仍然有距离，不过与临床结合思考，仍然有帮助的，不能仅仅是纯临床思维，转化思维同样重要, beContent=null, objectType=article, channel=null, level=null, likeNumber=50, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=肿肿, createdTime=Mon Jun 28 19:53:27 CST 2021, time=2021-06-28, status=1, ipAttribution=)]
    2021-06-28 肿肿

    机制研究离临床仍然有距离，不过与临床结合思考，仍然有帮助的，不能仅仅是纯临床思维，转化思维同样重要

    0

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