TY -的A2 Peltekian中m . AU - Thoeni科妮莉亚盟——Waldherr Ruediger盟——墙头草,任何人非盟- Schmitteckert,斯蒂芬妮盟——Roeth拉尔夫盟——Niesler Beate AU - Cutz欧内斯特盟——Flechtenmacher Christa盟——Goeppert本杰明盟——Schirmacher彼得盟——LasitschkaFelix PY - 2019 DA - 2019/12/10 TI -表达式分析腺苷结合盒转运蛋白ABCB11 ABCB4在原发性硬化性胆管炎和各种各样的儿童和成人淤胆型和Noncholestatic肝病SP - 1085717六世- 2019 AB -磷酸腺苷磁带(ABC)转运蛋白是射流泵的成员负责for the removal of cytotoxic substances by active transport. ABCB11, the bile salt efflux pump of hepatocytes, coordinates cellular excretion of numerous conjugated bile salts into the bile canaliculi, whereas ABCB4 acts as an ATP-dependent floppase translocating phosphatidylcholine from the inner to the outer leaflet of the bile canalicular membrane. Loss of functional ABCB11 and ABCB4 proteins causes early-onset refractory cholestasis or cholangiopathy. In this study, we investigated the expression and localization pattern of ABCB11 and ABCB4 using immunohistochemistry and RNA profiling in liver samples from patients with different types and stages of chronic cholestatic liver disease, with emphasis on primary sclerosing cholangitis (PSC), compared to a variety of cholestatic and noncholestatic hepatopathies. Therefore, ABCB11 and ABCB4 expressions were investigated on formalin-fixed and paraffin-embedded (FFPE) material in a patient cohort of total 43 patients with or without cholestatic liver diseases, on protein level using immunohistochemistry and on RNA level using nanoString technology. Intriguingly, our results demonstrated increased expression of ABCB11 and ABCB4 on protein as well as RNA level in PSC, and the expression pattern correlated with disease progression. We concluded from our study that patients with PSC demonstrate altered expression levels and pattern of ABCB11 and ABCB4 which correlated with disease progression; thereby, ABCB11 and ABCB4 analysis may be a useful tool for assessment of disease stages in PSC. SN - 2291-2789 UR - https://doi.org/10.1155/2019/1085717 DO - 10.1155/2019/1085717 JF - Canadian Journal of Gastroenterology and Hepatology PB - Hindawi KW - ER -