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Claudin-1 involved in neonatal ichthyosis sclerosing cholangitis syndrome regulates hepatic paracellular permeability

并行传输 克洛丹 紧密连接 生物 医学 鱼鳞病 先天性鱼鳞病 病理 细胞生物学 磁导率 内科学 皮肤病科 遗传学
作者
Brigitte Grosse,Doris Cassio,Nadya Yousef,Céline Bernardo,Emmanuel Jacquemin,Emmanuel Gonzalès
出处
期刊:Hepatology [Wiley]
卷期号:55 (4): 1249-1259 被引量:77
标识
DOI:10.1002/hep.24761
摘要

Abstract Neonatal ichthyosis and sclerosing cholangitis (NISCH) syndrome is a liver disease caused by mutations of CLDN1 encoding Claudin-1, a tight-junction (TJ) protein. In this syndrome, it is speculated that cholestasis is caused by Claudin-1 absence, leading to increased paracellular permeability and liver injuries secondary to paracellular bile regurgitation. We studied the role of claudin-1 in hepatic paracellular permeability. A NISCH liver and polarized rat cell lines forming TJs, the hepatocellular Can 10 and the cholangiocellular normal rat choloangiocyte (NRC), were used. In contrast to NRC, Can 10 does not express claudin-1. Can 10 cells were transfected with a plasmid encoding Claudin-1, and stable Claudin-1-expressing clones were isolated. Claudin-1 expression was silenced by transfection with short interfering RNA in Can 10 clones and with short hairpin RNA in NRC. Claudin-1 expression was evaluated by quantitative reverse-transcriptase polymerase chain reaction, immunoblotting, and immunolocalization. Paracellular permeability was assessed by fluorescein isothiocyanate-dextran passage in both lines and by transepithelial resistance measurements in NRC. In the NISCH liver, Claudin-1 was not detected in hepatocytes or cholangiocytes. In Claudin-1 expressing Can 10 clones, Claudin-1 was localized at the TJ and paracellular permeability was decreased, compared to parental Can 10 cells, this decrease correlating with claudin-1 levels. Silencing of Claudin-1 in Can 10 clones increased paracellular permeability to a level similar to that of parental cells. Similarly, we observed an increase of paracellular permeability in NRC cells silenced for claudin-1 expression. Conclusion : Defect in claudin-1 expression increases paracellular permeability in polarized hepatic cell lines, supporting the hypothesis that paracellular bile leakage through deficient TJs is involved in liver pathology observed in NISCH syndrome. (Hepatology 2012)
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