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  • 标题:Modeling lethal X-linked genetic disorders in pigs with ensured fertility
  • 本地全文:下载
  • 作者:Hitomi Matsunari ; Masahito Watanabe ; Kazuaki Nakano
  • 期刊名称:Proceedings of the National Academy of Sciences
  • 印刷版ISSN:0027-8424
  • 电子版ISSN:1091-6490
  • 出版年度:2018
  • 卷号:115
  • 期号:4
  • 页码:708-713
  • DOI:10.1073/pnas.1715940115
  • 语种:English
  • 出版社:The National Academy of Sciences of the United States of America
  • 摘要:Genetically engineered pigs play an indispensable role in the study of rare monogenic diseases. Pigs harboring a gene responsible for a specific disease can be efficiently generated via somatic cell cloning. The generation of somatic cell-cloned pigs from male cells with mutation(s) in an X chromosomal gene is a reliable and straightforward method for reproducing X-linked genetic diseases (XLGDs) in pigs. However, the severe symptoms of XLGDs are often accompanied by impaired growth and reproductive disorders, which hinder the reproduction of these valuable model animals. Here, we generated unique chimeric boars composed of mutant cells harboring a lethal XLGD and normal cells. The chimeric boars exhibited the cured phenotype with fertility while carrying and transmitting the genotype of the XLGD. This unique reproduction system permits routine production of XLGD model pigs through the male-based breeding, thereby opening an avenue for translational research using disease model pigs.
  • 关键词:blastocyst complementation ; chimera ; disease model pig ; gene knockout ; somatic cell cloning
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