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7/21/2021 8:24:10 AM | Browse: 203 | Download: 437
Publication Name World Journal of Clinical Cases
Manuscript ID 63810
Country/Territory China
Received
2021-02-03 08:57
Peer-Review Started
2021-02-03 09:02
To Make the First Decision
Return for Revision
2021-03-15 00:56
Revised
2021-03-22 09:01
Second Decision
2021-05-26 10:17
Accepted by Journal Editor-in-Chief
Accepted by Company Editor-in-Chief
2021-05-26 13:02
Articles in Press
2021-05-26 13:02
Publication Fee Transferred
Edit the Manuscript by Language Editor
2021-06-06 10:11
Typeset the Manuscript
2021-07-02 09:24
Publish the Manuscript Online
2021-07-21 08:24
ISSN 2307-8960 (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) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
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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 Observational Study
Article Title Potential protein–phenotype correlation in 3 LPS-responsive beige-like anchor protein-deficient patients
Manuscript Source Unsolicited Manuscript
All Author List Wen-Juan Tang, Wen-Hui Hu, Ying Huang, Bing-Bing Wu, Xiao-Min Peng, Xiao-Wen Zhai, Xiao-Wen Qian, Zi-Qing Ye, Hai-Jiao Xia, Jie Wu and Jie-Ru Shi
ORCID
Author(s) ORCID Number
Wen-Juan Tang http://orcid.org/0000-0001-8513-905X
Wen-Hui Hu http://orcid.org/0000-0002-6438-9559
Ying Huang http://orcid.org/0000-0002-9818-9803
Bing-Bing Wu http://orcid.org/0000-0001-9269-4414
Xiao-Min Peng http://orcid.org/0000-0002-1597-3783
Xiao-Wen Zhai http://orcid.org/0000-0002-8127-3232
Xiao-Wen Qian http://orcid.org/0000-0002-0072-6632
Zi-Qing Ye http://orcid.org/0000-0002-6588-2839
Hai-Jiao Xia http://orcid.org/0000-0001-7157-0738
Jie Wu http://orcid.org/0000-0002-6831-3981
Jie-Ru Shi http://orcid.org/0000-0002-3149-0466
Funding Agency and Grant Number
Corresponding Author Ying Huang, MD, PhD, Chief Doctor, Professor, Department of Gastroenterology, Pediatric Inflammatory Bowel Disease Research Center, Children’s Hospital of Fudan University, National Children's Medical Center, Wanyuan Road 399, Shanghai 201102, China. yhuang2019@126.com
Key Words LPS-responsive beige-like anchor protein deficiency; Chinese; Common variable immunodeficiency; Gene mutation; Chronic diarrhea
Core Tip Previous studies showed that there is no apparent genotype–phenotype correlation for LPS-responsive beige-like anchor protein (LRBA) deficiency, but may have protein–phenotype correlation. In this study we described 3 Chinese patients with LRBA deficiency, though all their major clinical findings indicated enteropathy, but they have different endoscopy findings and different response to the immunosuppressive treatment. Functional experiments revealed that a lack of LRBA protein expression may lead to worse disease outcomes and be an indicator for hematopoietic stem cell transplantation (HSCT). The results of this study will be valuable for the selection of an immunosuppressive treatment or HSCT for treating LRBA-deficient patients in the future.
Publish Date 2021-07-21 08:24
Citation Tang WJ, Hu WH, Huang Y, Wu BB, Peng XM, Zhai XW, Qian XW, Ye ZQ, Xia HJ, Wu J, Shi JR. Potential protein–phenotype correlation in 3 LPS-responsive beige-like anchor protein-deficient patients. World J Clin Cases 2021; 9(21): 5873-5888
URL https://www.wjgnet.com/2307-8960/full/v9/i21/5873.htm
DOI https://dx.doi.org/10.12998/wjcc.v9.i21.5873
Full Article (PDF) WJCC-9-5873.pdf
Full Article (Word) WJCC-9-5873.docx
Manuscript File 63810_Auto_Edited-ZMG_WangTQ.docx
Answering Reviewers 63810-Answering reviewers.pdf
Audio Core Tip 63810-Audio core tip.m4a
Biostatistics Review Certificate 63810-Biostatistics statement.pdf
Conflict-of-Interest Disclosure Form 63810-Conflict-of-interest statement.pdf
Copyright License Agreement 63810-Copyright license agreement.pdf
Signed Informed Consent Form(s) or Document(s) 63810-Informed consent statement.pdf
Institutional Review Board Approval Form or Document 63810-Institutional review board statement.pdf
Non-Native Speakers of English Editing Certificate 63810-Language certificate.pdf
Peer-review Report 63810-Peer-review(s).pdf
Scientific Misconduct Check 63810-Scientific misconduct check.pdf
Scientific Editor Work List 63810-Scientific editor work list.pdf