Laboratory investigation; a journal of technical methods and pathology · 2022
Generation of heterozygous PKD1 mutant pigs exhibiting early-onset renal cyst formation.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Watanabe Masahito, Umeyama Kazuhiro, Nakano Kazuaki, Matsunari Hitomi, Fukuda Toru, Matsumoto Kei, Tajiri Susumu, Yamanaka Shuichiro, Hasegawa Koki, Okamoto Kazutoshi, Uchikura Ayuko, Takayanagi Shuko, Nagaya Masaki, Yokoo Takashi, Nakauchi Hiromitsu, Nagashima Hiroshi
Аннотация
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease, manifesting as the progressive development of fluid-filled renal cysts. In approximately half of all patients with ADPKD, end-stage renal disease results in decreased renal function. In this study, we used CRISPR-Cas9 and somatic cell cloning to produce pigs with the unique mutation c.152_153insG (PKD1insG/+). Pathological analysis of founder cloned animals and progeny revealed that PKD1insG/+ pigs developed many pathological conditions similar to those of patients with heterozygous mutations in PKD1. Pathological similarities included the formation of macroscopic renal cysts at the neonatal stage, number and cystogenic dynamics of the renal cysts formed, interstitial fibrosis of the renal tissue, and presence of a premature asymptomatic stage. Our findings demonstrate that PKD1insG/+ pigs recapitulate the characteristic symptoms of ADPKD.