Jagged1 heterozygosity in mice results in a congenital cholangiopathy which is reversed by concomitant deletion of one copy of Poglut1 ( Rumi )
作者: Shakeel M. ThakurdasMario F. LopezShinako KakudaRodrigo Fernandez‐ValdiviaNeda Zarrin‐KhamehRobert S. HaltiwangerHamed Jafar‐Nejad
作者单位: 1Department of Molecular and Human Genetics Baylor College of Medicine Houston TX
2 Department of Biochemistry and Cell Biology Stony Brook University Stony Brook NY
3 Department of Pathology, School of Medicine Wayne State University Detroit MI
4 Department of Pathology and Immunology Baylor College of Medicine Houston TX
5 Program in Developmental Biology Baylor College of Medicine Houston TX
刊名: Hepatology, 2016, Vol.63 (2), pp.550-565
来源数据库: Wiley Journal
DOI: 10.1002/hep.28024
原始语种摘要: Haploinsufficiency for the Notch ligand JAG1 in humans results in an autosomal‐dominant, multisystem disorder known as Alagille syndrome, which is characterized by a congenital cholangiopathy of variable severity. Here, we show that on a C57BL/6 background, jagged1 heterozygous mice ( Jag1+/− ) exhibit impaired intrahepatic bile duct (IHBD) development, decreased SOX9 expression, and thinning of the periportal vascular smooth muscle cell (VSMC) layer, which are apparent at embryonic day 18 and the first postnatal week. In contrast, mice double heterozygous for Jag1 and the glycosyltransferase, Poglut1 ( Rumi ), start showing a significant improvement in IHBD development and VSMC differentiation during the first week. At P30, Jag1+/− mice show widespread ductular...
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影响因子:12.003 (2012)

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