斑马鱼异位矿化模型——弹性假黄瘤病的范例。
Zebrafish Models of Ectopic Mineralization-The Paradigm of Pseudoxanthoma Elasticum.
机构信息
Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA; PXE International Center of Excellence in Research and Clinical Care, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA; PXE International Center of Excellence in Research and Clinical Care, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
出版信息
J Invest Dermatol. 2018 Nov;138(11):2301-2304. doi: 10.1016/j.jid.2018.07.007.
Zebrafish represent a powerful model system with which to study human biology and pathology. Recently developed CRISPR/Cas9 technology enables genetic manipulation with precision. Using CRISPR/Cas9 methodology, van Gils et al. generated knockout zebrafish for abcc6a, the orthologue of human ABCC6 that is mutated in pseudoxanthoma elasticum. Although similarities exist between this and other abcc6a zebrafish models, none fully recapitulate phenotypes of human pseudoxanthoma elasticum.
斑马鱼是研究人类生物学和病理学的强大模型系统。最近开发的 CRISPR/Cas9 技术能够实现精确的基因操作。van Gils 等人利用 CRISPR/Cas9 方法，生成了 abcc6a 的敲除斑马鱼，abcc6a 的同源物在弹性假黄瘤中发生突变。尽管这种 abcc6a 斑马鱼模型与其他模型存在相似之处，但没有一个能完全重现人类弹性假黄瘤的表型。
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