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Article: Deletion of interleukin enhancer binding factor 2 (ILF2) resulted in defective biliary development and bile flow blockage

TitleDeletion of interleukin enhancer binding factor 2 (ILF2) resulted in defective biliary development and bile flow blockage
Authors
KeywordsLiver
Bile duct
Biliary atresia
ILF2
Zebrafish
Issue Date2021
PublisherWB Saunders Co. The Journal's web site is located at http://www.elsevier.com/locate/jpedsurg
Citation
Journal of Pediatric Surgery, 2021, v. 56 n. 2, p. 352-359 How to Cite?
AbstractPurpose Biliary atresia (BA) is a devastating obstructive bile duct disease of newborns. BA has the highest incidence in Asians (1/5000), and its pathogenesis is unclear. We identified BA-private rare copy number variants (CNVs; 22 duplications and 6 deletions). ILF2 gene locates in the chromosome region (Chr1:153410347–153,634,058) which was deleted in a nonsyndromic BA patient. However, it is still not known whether ILF2 plays a role in hepatobiliary development and its deletion impacts on the bile duct development. Methods To investigate if ILF2 is required for biliary development, we knock-out the zebrafish homologs of ILF2 by CRISPR/Cas9 approach, and discover that deletion of ILF2 causes a defective biliary development and a lack of bile flow from the liver to the gall bladder in zebrafish, which is a resemblance of phenotypes of BA. Results Our data indicate that ILF2 gene is required for biliary development; deletion of ILF2 impairs bile duct development and could contribute to BA pathogenesis. This will be the first study to functionally evaluate the genes interfered by BA-private CNVs in hepatobiliary development and in BA pathogenesis. Conclusions Such functional study may reveal the potential value of these BA-private CNVs in the disease pathogenesis for BA. Level of evidence N/A (animal and laboratory study).
DescriptionHybrid open access
Persistent Identifierhttp://hdl.handle.net/10722/296377
ISSN
2023 Impact Factor: 2.4
2023 SCImago Journal Rankings: 0.949
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorCheung, Y-
dc.contributor.authorWu, Z-
dc.contributor.authorGarcia-Barcelo, MM-
dc.contributor.authorTam, PKH-
dc.contributor.authorMa, ACH-
dc.contributor.authorLui, VCH-
dc.date.accessioned2021-02-22T04:54:25Z-
dc.date.available2021-02-22T04:54:25Z-
dc.date.issued2021-
dc.identifier.citationJournal of Pediatric Surgery, 2021, v. 56 n. 2, p. 352-359-
dc.identifier.issn0022-3468-
dc.identifier.urihttp://hdl.handle.net/10722/296377-
dc.descriptionHybrid open access-
dc.description.abstractPurpose Biliary atresia (BA) is a devastating obstructive bile duct disease of newborns. BA has the highest incidence in Asians (1/5000), and its pathogenesis is unclear. We identified BA-private rare copy number variants (CNVs; 22 duplications and 6 deletions). ILF2 gene locates in the chromosome region (Chr1:153410347–153,634,058) which was deleted in a nonsyndromic BA patient. However, it is still not known whether ILF2 plays a role in hepatobiliary development and its deletion impacts on the bile duct development. Methods To investigate if ILF2 is required for biliary development, we knock-out the zebrafish homologs of ILF2 by CRISPR/Cas9 approach, and discover that deletion of ILF2 causes a defective biliary development and a lack of bile flow from the liver to the gall bladder in zebrafish, which is a resemblance of phenotypes of BA. Results Our data indicate that ILF2 gene is required for biliary development; deletion of ILF2 impairs bile duct development and could contribute to BA pathogenesis. This will be the first study to functionally evaluate the genes interfered by BA-private CNVs in hepatobiliary development and in BA pathogenesis. Conclusions Such functional study may reveal the potential value of these BA-private CNVs in the disease pathogenesis for BA. Level of evidence N/A (animal and laboratory study).-
dc.languageeng-
dc.publisherWB Saunders Co. The Journal's web site is located at http://www.elsevier.com/locate/jpedsurg-
dc.relation.ispartofJournal of Pediatric Surgery-
dc.rightsThis work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.-
dc.subjectLiver-
dc.subjectBile duct-
dc.subjectBiliary atresia-
dc.subjectILF2-
dc.subjectZebrafish-
dc.titleDeletion of interleukin enhancer binding factor 2 (ILF2) resulted in defective biliary development and bile flow blockage-
dc.typeArticle-
dc.identifier.emailWu, Z: hannawu@hku.hk-
dc.identifier.emailGarcia-Barcelo, MM: mmgarcia@hku.hk-
dc.identifier.emailTam, PKH: paultam@hku.hk-
dc.identifier.emailLui, VCH: vchlui@hku.hk-
dc.identifier.authorityGarcia-Barcelo, MM=rp00445-
dc.identifier.authorityTam, PKH=rp00060-
dc.identifier.authorityLui, VCH=rp00363-
dc.description.naturepublished_or_final_version-
dc.identifier.doi10.1016/j.jpedsurg.2020.06.032-
dc.identifier.pmid32709532-
dc.identifier.scopuseid_2-s2.0-85088219243-
dc.identifier.hkuros321293-
dc.identifier.volume56-
dc.identifier.issue2-
dc.identifier.spage352-
dc.identifier.epage359-
dc.identifier.isiWOS:000616821600027-
dc.publisher.placeUnited States-

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