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Oral Dis：研究揭示颅骨发育不良患者牙囊细胞骨重建异常的原因

2018-06-23 MedSci MedSci原创

颅骨发育不良牙囊细胞骨重建
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本研究旨在探讨新型颅骨发育不良（CCD）致病基因RUNX2基因突变（DFCsRUNX2 +/m）在恒牙延迟萌出中的作用。 纳入具有典型临床特征的CCD患者。培养DFCsRUNX2 +/m并提取DNA用于RUNX2突变筛选。细胞增殖、碱性磷酸酶（ALP）活性、茜素红染色和成骨细胞特异性基因表达的测量以评估DFCsRUNX2 +/m的成骨作用。抗酒石酸酸性磷酸酶（TRAP）染色，real-time P

本研究旨在探讨新型颅骨发育不良（CCD）致病基因RUNX2基因突变（DFCsRUNX2 +/m）在恒牙延迟萌出中的作用。

纳入具有典型临床特征的CCD患者。培养DFCsRUNX2 +/m并提取DNA用于RUNX2突变筛选。细胞增殖、碱性磷酸酶（ALP）活性、茜素红染色和成骨细胞特异性基因表达的测量以评估DFCsRUNX2 +/m的成骨作用。抗酒石酸酸性磷酸酶（TRAP）染色，real-time PCR和Western blot方法检测DFCs和外周血单个核细胞（PBMCs）的共培养，评估DFCsRUNX2 +/m的破骨细胞诱导能力。

结果显示，CCD患者的DFCsRUNX2 +/m中出现错义RUNX2突变（c.557G>C）。与正常对照组相比，该突变不影响DFCsRUNX2 +/m的增殖，但下调成骨相关基因的表达，导致ALP活性和矿化降低。共培养结果显示DFCsRUNX2 + / m减少了TRAP +多核细胞的形成和破骨细胞生成相关基因的表达。此外，该突变降低了DFCsRUNX2 +/m中RANKL/OPG的比率。

综上所述，该研究结果表明，DFCsRUNX2 +/m通过RANK/RANKL/OPG信号传导通路干扰牙齿萌出过程中的骨重塑活动，因此可能导致CCD患者永久性牙齿萌出受损。

原始出处：

Liu Y, Zhang X, et al., Abnormal bone remodelling activity of dental follicle cells from a cleidocranial dysplasia patient. Oral Dis. 2018 May 22. doi: 10.1111/odi.12900.

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    2018-12-15 cmj8wellington

    #Dis#

    0

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    2019-02-08 智慧医人

    #骨重建#

    0

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    2018-09-12 小几洁

    #Oral#

    0

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    2018-06-25 neurowu

    #发育#

    0

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    2018-06-25 villahu

    #发育不良#

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=2012716, encodeId=9a2c2012e16e6, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=68, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Sat Dec 15 06:35:00 CST 2018, time=2018-12-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1710490, encodeId=7b291e10490e7, content=<a href='/topic/show?id=ecec10189e7b' target=_blank style='color:#2F92EE;'>#骨重建#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=104, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=101897, encryptionId=ecec10189e7b, topicName=骨重建)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智慧医人, createdTime=Fri Feb 08 07:35:00 CST 2019, time=2019-02-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2020437, encodeId=dc62202043e38, content=<a href='/topic/show?id=1584134451c' target=_blank style='color:#2F92EE;'>#Oral#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=57, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13445, encryptionId=1584134451c, topicName=Oral)], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIpeueeYB1kem1yfEZrSYKBdZBoqac9USf0RxIgRICBIyDn4licyiaiakNnobeCDVpvwqaWgwTNeLRHA/132, createdBy=bb5e2500127, createdName=小几洁, createdTime=Wed Sep 12 07:35:00 CST 2018, time=2018-09-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1347309, encodeId=b13f134e309c1, content=<a href='/topic/show?id=88713e938c1' target=_blank style='color:#2F92EE;'>#发育#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=77, 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=Mon Jun 25 08:35:00 CST 2018, time=2018-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1567970, encodeId=3521156e97059, content=<a href='/topic/show?id=7a833e94079' target=_blank style='color:#2F92EE;'>#发育不良#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37940, encryptionId=7a833e94079, topicName=发育不良)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=30b515313716, createdName=villahu, createdTime=Mon Jun 25 08:35:00 CST 2018, time=2018-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=326394, encodeId=de0332639499, content=学习了.长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=117, 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=Sat Jun 23 18:43:13 CST 2018, time=2018-06-23, status=1, ipAttribution=)]
    2018-06-23 1ddf0692m34(暂无匿称)

    学习了.长知识

    0

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