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

doi:10.3969/j.issn.1001-5256.2022.05.041

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

杨玉龙¹,
魏涛华²,
杨文明^{2, 3, , ,},
郝文杰¹,
杨悦¹,
钱南南¹,
李祥²,
江海林²

1.
安徽中医药大学研究生院，合肥 230000
2.
安徽中医药大学第一附属医院脑病中心，合肥 230000
3.
新安医学教育部重点实验室，合肥 230000

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

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出版历程
- 收稿日期: 2021-09-01
- 出版日期: 2022-05-20

Research advances in animal models of Wilson's disease

1.
Graduate School of Anhui University of Chinese Medicine, Hefei 230000, China
2.
Encephalopathy Center, The First Affiliated Hospital of Anhui University of Chinese Medicine, Hefei 230000, China
3.
Key Laboratory of Xin'an Medicine, Ministry of Education, Hefei 230000, China

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

Corresponding author: YANG Wenming, yangwm8810@126.com(ORCID: 0000-0001-8268-9291)

摘要

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。
- 肝豆状核变性 /
- 肝疾病 /
- 疾病模型, 动物
Abstract: Wilson's disease (WD) is a rare autosomal recessive disorder with a complex pathogenesis involving multiple systems, multiple visceral organs, and the complex copper homeostasis regulation system within the body. The liver is the most common organ for copper deposition, and liver injury is the earliest and most common manifestation of WD; therefore, it is important to find an ideal animal model for WD research. By summarizing the animal models of WD commonly used in the world, this article systematically summarizes the background, liver and nervous manifestations, and application of different models and compares the characteristics of different animal models, so as to provide a reference for the application of various animal models of WD.
- Hepatolenticular Degeneration /
- Liver Diseases /
- Disease Models, Animal

HTML全文

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	有	无	无	无	肝损伤出现较早且表现突出，应用较广泛
TX-J小鼠	有	轻微	无	无	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	有	无	无	无	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	有	轻微	无	有	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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