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

白细胞介素增强子结合因子2 (ILF2) 的缺失导致胆道发育缺陷和胆汁流动阻塞。

  • 影响因子:2.14
  • DOI:10.1016/j.jpedsurg.2020.06.032
  • 作者列表:"Cheung Y","Wu Z","Garcia-Barcelo MM","Tam PKH","Ma ACH","Lui VCH
  • 发表时间:2021-02-01
Abstract

PURPOSE: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).

摘要

目的: 胆道闭锁 (BA) 是一种严重的新生儿胆道梗阻性疾病。BA在亚洲人中发病率最高 (1/5000),其发病机制尚不清楚。我们鉴定了BA-私人罕见拷贝数变异 (CNVs; 22个重复和6个缺失)。ILF2基因位于染色体区域 (Chr1:153410347-153,634,058),在非综合征型BA患者中缺失。然而,ILF2是否在肝胆发育中发挥作用以及其缺失对胆管发育的影响尚不清楚。 方法: 为了研究胆汁发育是否需要ILF2,我们通过CRISPR/Cas9方法敲除ILF2的斑马鱼同源物,并发现ILF2的缺失会导致胆汁发育缺陷和斑马鱼肝脏至胆囊的胆汁流不足,这是BA表型的相似之处。 结果: 我们的数据表明ILF2基因是胆道发育所必需的; ILF2的缺失会损害胆管发育,并可能导致BA发病。这将是第一项在功能上评估在肝胆发育和BA发病机制中受BA-私人cnv干扰的基因的研究。 结论: 此类功能研究可能揭示这些BA-私人cnv在BA的疾病发病机制中的潜在价值。 证据级别: N/A (动物和实验室研究)。

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DOI:10.1007/s11033-021-06299-9
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