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Articles Published Processes
12/19/2020 11:55:31 AM | Browse: 654 | Download: 1244
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Received |
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2020-09-11 19:33 |
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Peer-Review Started |
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2020-09-11 19:34 |
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To Make the First Decision |
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Return for Revision |
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2020-10-27 17:26 |
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Revised |
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2020-11-05 15:52 |
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Second Decision |
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2020-11-27 08:36 |
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Accepted by Journal Editor-in-Chief |
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Accepted by Executive Editor-in-Chief |
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2020-11-29 04:09 |
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Articles in Press |
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2020-11-29 04:09 |
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Publication Fee Transferred |
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Edit the Manuscript by Language Editor |
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Typeset the Manuscript |
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2020-12-17 02:54 |
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Publish the Manuscript Online |
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2020-12-19 11:55 |
| ISSN |
1007-9327 (print) and 2219-2840 (online) |
| Open Access |
This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/ |
| Copyright |
© The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved. |
| Article Reprints |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/247
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| Permissions |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/207
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| Publisher |
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA |
| Website |
http://www.wjgnet.com |
| Category |
Gastroenterology & Hepatology |
| Manuscript Type |
Minireviews |
| Article Title |
Molecular overview of progressive familial intrahepatic cholestasis
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| Manuscript Source |
Invited Manuscript |
| All Author List |
Sriram Amirneni, Nils Haep, Mohammad A Gad, Alejandro Soto-Gutierrez, James E Squires and Rodrigo M Florentino |
| ORCID |
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| Funding Agency and Grant Number |
| Funding Agency |
Grant Number |
| NIH |
UG3TR003289 |
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| Corresponding Author |
Rodrigo M Florentino, PhD, Research Fellow, Department of Pathology, University of Pittsburgh, 200 Lothrop Street S420 Biomedical Science Tower
, Pittsburgh, PA 15213, United States. rodrigomachado@pitt.edu |
| Key Words |
progressive familial intrahepatic cholestasis; ATP8B1/familial intrahepatic cholestasis 1; ABCB11/bile salt export pump; ABCB4/multidrug resistance class 3; Intrahepatic cholestasis; Bile |
| Core Tip |
Progressive familial intrahepatic cholestasis (PFIC) is an expanding group of genetically-based diseases caused by various autosomal recessive mutations which result in the inability to appropriately form and excrete bile from hepatocytes. There are three classic types of PFIC. Type 1 is caused by mutations of the ATP8B1 gene, encoding familial intrahepatic cholestasis 1. PFIC 2 is the result of mutations in the ABCB11 gene, reducing the apical membrane expression of bile salt export pump. PFIC 3 is caused by variations in the gene that encodes the multidrug resistance class 3 glycoprotein. With advances in DNA sequencing, new forms of this disease have been documented. Alterations in the NR1H4, tight junction protein 2, and MYO5B genes have been linked with new phenotypes of PFIC. Here, we provide a molecular and clinical overview of PFIC. |
| Publish Date |
2020-12-19 11:55 |
| Citation |
Amirneni S, Haep N, Gad MA, Soto-Gutierrez A, Squires JE, Florentino RM. Molecular overview of progressive familial intrahepatic cholestasis. World J Gastroenterol 2020; 26(47): 7470-7484 |
| URL |
https://www.wjgnet.com/1007-9327/full/v26/i47/7470.htm |
| DOI |
https://dx.doi.org/10.3748/wjg.v26.i47.7470 |
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