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Comp Med. 2014 Apr;64(2):115-20.
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本文引用的文献

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Systematic review: the pathophysiology and management of polycystic liver disease.系统综述:多囊肝疾病的病理生理学和治疗管理。
Aliment Pharmacol Ther. 2011 Oct;34(7):702-13. doi: 10.1111/j.1365-2036.2011.04783.x. Epub 2011 Jul 26.
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Polycystic liver diseases: congenital disorders of cholangiocyte signaling.多囊肝疾病:胆管细胞信号传导的先天性疾病。
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Proliferative and nonproliferative lesions of the rat and mouse hepatobiliary system.大鼠和小鼠肝胆系统的增殖性和非增殖性病变
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Polycystic liver diseases.多囊性肝病。
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Polycystin-1 and -2 dosage regulates pressure sensing.多囊蛋白-1和-2的剂量调节压力感知。
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Liver and kidney disease in ciliopathies.纤毛病中的肝和肾病。
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Ciliary and centrosomal defects associated with mutation and depletion of the Meckel syndrome genes MKS1 and MKS3.与梅克尔综合征基因MKS1和MKS3的突变及缺失相关的纤毛和中心体缺陷
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Mouse models of polycystic kidney disease.多囊肾病的小鼠模型。
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Mutation of murine adenylate kinase 7 underlies a primary ciliary dyskinesia phenotype.小鼠腺苷酸激酶7的突变是原发性纤毛运动障碍表型的基础。
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10
Overexpression of RPGR leads to male infertility in mice due to defects in flagellar assembly.RPGR的过表达由于鞭毛组装缺陷导致小鼠雄性不育。
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案例研究:转基因小鼠品系中的多囊肝

Case study: polycystic livers in a transgenic mouse line.

作者信息

Lovaglio Jamie, Artwohl James E, Ward Christopher J, Diekwisch Thomas Gh, Ito Yoshihiro, Fortman Jeffrey D

机构信息

Biologic Resources Laboratory, University of Illinois at Chicago, Chicago, Illinois, USA.

Mayo Clinic, Rochester, Maine, USA.

出版信息

Comp Med. 2014 Apr;64(2):115-20.

PMID:24674586
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3997289/
Abstract

Three mice (2 male, 1 female; age, 5 to 16 mo) from a mouse line transgenic for keratin 14 (K14)-driven LacZ expression and on an outbred Crl:CD1(ICR) background, were identified as having distended abdomens and livers that were diffusely enlarged by numerous cysts (diameter, 0.1 to 2.0 cm). Histopathology revealed hepatic cysts lined by biliary type epithelium and mild chronic inflammation, and confirmed the absence of parasites. Among 21 related mice, 5 additional affected mice were identified via laparotomy. Breeding of these 5 mice (after 5 mo of age) did not result in any offspring; the K14 mice with polycystic livers failed to reproduce. Affected male mice had degenerative testicular lesions, and their sperm was immotile. Nonpolycystic K14 control male mice bred well, had no testicular lesions, and had appropriate sperm motility. Genetic analysis did not identify an association of this phenotype with the transgene or insertion site.

摘要

来自一个角蛋白14(K14)驱动LacZ表达的转基因小鼠品系、背景为远交Crl:CD1(ICR)的三只小鼠(2只雄性,1只雌性;年龄5至16个月),被发现腹部膨隆,肝脏因大量囊肿(直径0.1至2.0厘米)而弥漫性肿大。组织病理学显示肝囊肿内衬胆管型上皮,伴有轻度慢性炎症,并证实无寄生虫。在21只相关小鼠中,通过剖腹术又发现了5只患病小鼠。这5只小鼠(5月龄后)繁殖未产生任何后代;患有多囊肝的K14小鼠无法繁殖。患病雄性小鼠有睾丸退行性病变,其精子无运动能力。非多囊性K14对照雄性小鼠繁殖良好,无睾丸病变,精子运动能力正常。基因分析未发现该表型与转基因或插入位点存在关联。