Ahn Sung-Hoon, Shah Yatrik M, Inoue Junko, Morimura Keiichirou, Kim Insook, Yim Sunhee, Lambert Gilles, Kurotani Reiko, Nagashima Kunio, Gonzalez Frank J, Inoue Yusuke
Laboratory of Metabolism, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Inflamm Bowel Dis. 2008 Jul;14(7):908-20. doi: 10.1002/ibd.20413.
Hepatocyte nuclear factor 4alpha (HNF4alpha; NR2A1) is an orphan member of the nuclear receptor superfamily expressed in liver and intestine. While HNF4alpha expression is critical for liver function, its role in the gut and in the pathogenesis of inflammatory bowel disease (IBD) is unknown.
Human intestinal biopsies from control and IBD patients were examined for expression of mRNAs encoding HNF4alpha and other nuclear receptors. An intestine-specific HNF4alpha null mouse line (Hnf4alpha(DeltaIEpC)) was generated using an Hnf4alpha-floxed allele and villin-Cre transgene. These mice and their control floxed counterparts (Hnf4alpha(F/F)), were subjected to a dextran sulfate sodium (DSS)-induced IBD colitis protocol and their clinical symptoms and gene expression patterns determined.
In human intestinal biopsies, HNF4alpha was significantly decreased in intestinal tissues from Crohn's disease and ulcerative colitis patients. HNF4alpha expression was also suppressed in the intestine of DSS-treated mice. In Hnf4alpha(DeltaIEpC) mice, disruption of HNF4alpha expression was observed in the epithelial cells throughout the intestine. In the DSS-induced colitis model Hnf4alpha(DeltaIEpC) mice showed markedly more severe changes in clinical symptoms and pathologies associated with IBD including loss of body weight, colon length, and histological morphology as compared with Hnf4alpha(F/F) mice. Furthermore, the Hnf4alpha(DeltaIEpC) mice demonstrate a significant alteration of mucin-associated genes and increased intestinal permeability, which may play an important role in the increased susceptibility to acute colitis following an inflammatory insult.
While HNF4alpha does not have a major role in normal function of the intestine, it protects the gut against DSS-induced colitis.
肝细胞核因子4α(HNF4α;NR2A1)是核受体超家族的一个孤儿成员,在肝脏和肠道中表达。虽然HNF4α的表达对肝功能至关重要,但其在肠道及炎症性肠病(IBD)发病机制中的作用尚不清楚。
检测对照患者和IBD患者的人肠道活检组织中编码HNF4α和其他核受体的mRNA表达。使用Hnf4α-floxed等位基因和绒毛蛋白-Cre转基因构建肠道特异性HNF4α敲除小鼠品系(Hnf4alpha(DeltaIEpC))。将这些小鼠及其对照的floxed同窝小鼠(Hnf4alpha(F/F))进行葡聚糖硫酸钠(DSS)诱导的IBD结肠炎实验方案处理,并确定其临床症状和基因表达模式。
在人肠道活检组织中,克罗恩病和溃疡性结肠炎患者的肠道组织中HNF4α显著降低。DSS处理小鼠的肠道中HNF4α表达也受到抑制。在Hnf4alpha(DeltaIEpC)小鼠中,在整个肠道的上皮细胞中观察到HNF4α表达的破坏。在DSS诱导的结肠炎模型中,与Hnf4alpha(F/F)小鼠相比,Hnf4alpha(DeltaIEpC)小鼠在临床症状和与IBD相关的病理变化方面表现出明显更严重的改变,包括体重减轻、结肠长度和组织形态学变化。此外,Hnf4alpha(DeltaIEpC)小鼠表现出粘蛋白相关基因的显著改变和肠道通透性增加,这可能在炎症刺激后对急性结肠炎易感性增加中起重要作用。
虽然HNF4α在肠道正常功能中没有主要作用,但它可保护肠道免受DSS诱导的结肠炎。