The phospholipid flippase ATP8B1 mediates apical localization of the cystic fibrosis transmembrane regulator
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- 作者：Vincent A. van der Marka ; Hugo R. de Jongeb ; Jung-Chin Changa ; Kam S. Ho-Moka ; Suzanne Duijsta ; Dragana Vidovićc ; Marianne S. Carlonc ; Ronald P.J. Oude Elferinka ; Coen C. Paulusmaa ; c.c.paulusma@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author
- 关键词：PFIC1 ; progressive familial intrahepatic cholestasis type 1 ; CFTR ; cystic fibrosis transmembrane conductance regulator ; CF ; cystic fibrosis ; Isc ; short circuit current ; TGN ; trans-Golgi network ; ASBT ; apical sodium-dependent bile acid transporter ; AP ; alkaline phosphatase ; SI ; sucrose isomaltase ; APN ; aminopeptidase N
- 刊名：Biochimica et Biophysica Acta (BBA)-Molecular Cell Research
- 出版年：2016
- 出版时间：September 2016
- 年：2016
- 卷：1863
- 期：9
- 页码：2280-2288
- 全文大小：901 K
文摘
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ATP8B1 depletion reduces CFTR mRNA levels in intestinal and pulmonary cells.
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ATP8B1 depletion reduces CFTR surface expression in intestinal and pulmonary cells.
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CFTR overexpression in ATP8B1-depleted cells does not restore its surface expression.
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ATP8B1 deficiency in mice does not affect CFTR expression nor activity.