Division of Gastroenterology, Department of Internal Medicine, University of Michigan, Ann Arbor, 48109-2200, USA.
Am J Physiol Gastrointest Liver Physiol. 2012 Sep 15;303(6):G752-64. doi: 10.1152/ajpgi.00109.2012. Epub 2012 Jul 5.
Antral gastrin is the hormone known to stimulate acid secretion and proliferation of the gastric corpus epithelium. Patients with mutations in the multiple endocrine neoplasia type 1 (MEN1) locus, which encodes the protein menin, develop pituitary hyperplasia, insulinomas, and gastrinomas in the duodenum. We previously hypothesized that loss of menin leads to derepression of the gastrin gene and hypergastrinemia. Indeed, we show that menin represses JunD induction of gastrin in vitro. Therefore, we examined whether conditional deletion of Men1 (Villin-Cre and Lgr5-EGFP-IRES-CreERT2), with subsequent loss of menin from the gastrointestinal epithelium, increases gastrin expression. We found that epithelium-specific deletion of Men1 using Villin-Cre increased plasma gastrin, antral G cell numbers, and gastrin expression in the antrum, but not the duodenum. Moreover, the mice were hypochlorhydric by 12 mo of age, and gastric somatostatin mRNA levels were reduced. However, duodenal mRNA levels of the cyclin-dependent kinase inhibitor p27(Kip1) were decreased, and cell proliferation determined by Ki67 staining was increased. About 11% of the menin-deficient mice developed antral tumors that were negative for gastrin; however, gastrinomas were not observed, even at 12 mo of age. No gastrinomas were observed with conditional deletion of Men1 in the Lgr5 stem cells 5 mo after Cre induction. In summary, epithelium-specific deletion of the Men1 locus resulted in hypergastrinemia due to antral G cell hyperplasia and a hyperproliferative epithelium, but no gastrinomas. This result suggests that additional mutations in gene targets other than the Men1 locus are required to produce gastrin-secreting tumors.
胃窦胃泌素是一种已知能刺激胃酸分泌和胃体上皮细胞增殖的激素。患有多发性内分泌肿瘤 1 型(MEN1)基因突变的患者,该基因突变编码 menin 蛋白,会在十二指肠中发展为垂体增生、胰岛素瘤和胃泌素瘤。我们之前假设 menin 的缺失会导致胃泌素基因去抑制和高胃泌素血症。事实上,我们表明 menin 抑制 JunD 诱导的胃泌素体外表达。因此,我们检查了胃肠道上皮细胞中 Men1(Villin-Cre 和 Lgr5-EGFP-IRES-CreERT2)的条件性缺失,随后 menin 从胃肠道上皮细胞中丢失,是否会增加胃泌素的表达。我们发现,使用 Villin-Cre 特异性缺失 Men1 会增加血浆胃泌素、胃窦 G 细胞数量和胃窦胃泌素表达,但不会增加十二指肠胃泌素表达。此外,这些小鼠在 12 月龄时胃酸分泌减少,胃生长抑素 mRNA 水平降低。然而,十二指肠 cyclin-dependent kinase inhibitor p27(Kip1)的 mRNA 水平降低,Ki67 染色测定的细胞增殖增加。约 11%的 menin 缺失小鼠发展为胃窦肿瘤,这些肿瘤对胃泌素呈阴性;然而,即使在 12 月龄时,也未观察到胃泌素瘤。在 Cre 诱导后 5 个月,Lgr5 干细胞中 Men1 条件性缺失未观察到胃泌素瘤。总之,Men1 基因座的上皮细胞特异性缺失导致胃窦 G 细胞增生和上皮过度增殖引起的高胃泌素血症,但没有胃泌素瘤。这一结果表明,需要 MEN1 基因座以外的其他基因靶标的额外突变才能产生分泌胃泌素的肿瘤。