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肝豆状核变性动物模型的研究进展

杨玉龙 魏涛华 杨文明 郝文杰 杨悦 钱南南 李祥 江海林

引用本文:
Citation:

肝豆状核变性动物模型的研究进展

DOI: 10.3969/j.issn.1001-5256.2022.05.041
基金项目: 

国家自然科学基金 (81973825)

国家中医药脑病循证能力提升及平台建设项目 (2019XZZX-NB001)

安徽中医药大学新安医学教育部重点实验室开放基金项目 (2020xayx12)

安徽高校协同创新项目 (GXXT-2020-025)

安徽省自然科学基金青年资助项目 (2108085QH367)

利益冲突声明:所有作者均声明不存在利益冲突。
作者贡献声明:杨玉龙、魏涛华负责课题设计,撰写论文; 郝文杰、杨悦、钱南南、李祥、江海林参与收集数据,修改论文; 杨文明负责拟定写作思路,指导撰写文章并最后定稿。
详细信息
    通信作者:

    杨文明,yangwm8810@126.com

Research advances in animal models of Wilson's disease

Research funding: 

National Natural Science Foundation of China (81973825);

National Project to Improve the Evidence-based Ability and Platform for TCM Encephalopathy (2019XZZX-NB001);

Open Foundation Project of Key Laboratory of Xin'an Medical Science, Ministry of Education, Anhui University of Chinese Medicine (2020xayx12);

The University Synergy Innovation Program of Anhui Province (GXXT-2020-025);

Anhui Provincial Natural Science Foundation Youth Funding Project (2108085QH367)

More Information
  • 摘要: 肝豆状核变性(WD)是一种罕见的常染色体隐性遗传病,其发病机制复杂,涉及多系统多脏器及体内复杂的铜稳态调节系统,其中肝脏是铜离子最常沉积的器官,肝损伤也是WD最早和最常见的表现,因此寻找一种理想的动物模型在WD研究中非常重要。本文通过对目前国际上常用的WD动物模型进行综述,系统地归纳了不同模型的背景,肝脏、神经等系统表现以及模型应用,并对不同动物模型的特点进行了比较,为各类WD动物模型的应用提供借鉴。

     

  • 表  1  常见WD动物模型特点

    Table  1.   Characteristics of common WD animal models

    模型种类 肝损伤
    症状
    神经系统症状
    (无/轻微/明显)
    K-F环
    (有/无)
    母乳中是否含
    有铜(有/无)
    应用特点
    TX小鼠 肝损伤出现较早且表现突出,应用较广泛
    TX-J小鼠 轻微 铜沉积出现较早,适合进行铜代谢等方面研究,应用较广
    ATP7B-/-小鼠 肝铜沉积较早且含量较高,适用于疗效评估,但价格昂贵,目前应用尚不广泛
    LEC大鼠 轻微 肝病进展迅速,适合用于进行干预研究,但大鼠病死率较高
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  • 收稿日期:  2021-09-01
  • 出版日期:  2022-05-20
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