Mouse models for blistering skin disorders.
Mouse models for blistering skin disorders.
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DOI：
10.1155/2010/584353
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发表时间：
2010
影响因子：
1.5
通讯作者：
Koch PJ
中科院分区：
文献类型：
--
作者：
Ganeshan R;Chen J;Koch PJ
基因工程小鼠一直是阐明人类疾病潜在病理机制的重要工具。在桥粒功能受损引起的疾病中，小鼠模型帮助建立了突变和疾病表型之间的因果联系。这篇综述集中于在复层上皮中缺乏桥粒钙粘附素、桥粒粘附素3或桥粒粘附素3的小鼠。提供了在这些小鼠品系中观察到的表型的比较，并讨论了突变的小鼠表型与人类疾病，特别是寻常型天疱疮之间的关系。此外，我们还将讨论基因工程小鼠品系在了解水疱性皮肤病方面的优势和潜在局限性。
Genetically engineered mice have been essential tools for elucidating the pathological mechanisms underlying human diseases. In the case of diseases caused by impaired desmosome function, mouse models have helped to establish causal links between mutations and disease phenotypes. This review focuses on mice that lack the desmosomal cadherins desmoglein 3 or desmocollin 3 in stratified epithelia. A comparison of the phenotypes observed in these mouse lines is provided and the relationship between the mutant mouse phenotypes and human diseases, in particular pemphigus vulgaris, is discussed. Furthermore, we will discuss the advantages and potential limitations of genetically engineered mouse lines in our ongoing quest to understand blistering skin diseases.