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肝细胞核因子-4与MODY 被引量：4

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摘要 肝细胞核因子 4(HNF 4)是高度保守的孤受体家族成员之一。载脂蛋白、α1抗胰蛋白酶、丙酮酸激酶和红细胞生成素等编码基因启动子中均含有HNF 4反应元件。HNF 4可与其他HNF分子共同组成HNFs转录因子调节网络 ,并可与HNF 4反应元件作用 ,进而调节组织分化、糖及脂类等能量物质的代谢。HNF 4的突变型可诱发青少年发病型成人糖尿病 (MODY)。对HNF 4α的无义突变型HNF 4.Q2 6 8X和HNF 4.E2 76Q的研究揭示出MODY形成的分子机制 ,为MODY的基因治疗奠定了理论基础。
作者 宋景春 李志超 张南雁 吴元明
机构地区 第四军医大学病理生理学教研室 第四军医大学西京医院内分泌科 第四军医大学生化教研室
出处 《国外医学（内分泌学分册）》 2001年第1期13-15,共3页 Foreign Medical Sciences(Section of Endocrinology)
关键词 HNF-4 HNF-4反应元件 HNFS调节网络 MODY1 病理
分类号 R392.12 [医药卫生—免疫学]
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参考文献10

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同被引文献59

  • 1何明坤,陆付耳,王开富,冷三华,徐丽君,邹欣.小檗碱对高脂血症伴胰岛素抵抗大鼠糖脂代谢的影响[J].中国医院药学杂志,2004,24(7):389-391. 被引量：34
  • 2吴静,张素华,倪银星.2型糖尿病大鼠肝脏肝细胞核因子(HNF)-4α、HNF-1α基因表达研究[J].中华内分泌代谢杂志,2005,21(4):325-326. 被引量：5
  • 3欧阳礼枝,陆付耳,刘文军,高志强,徐丽君.小檗碱对胰岛素抵抗大鼠肝脏葡萄糖激酶及其调节蛋白的影响[J].世界华人消化杂志,2007,15(8):885-889. 被引量：17
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  • 5Barrio R,Bellanne-Chantelot C,Moreno J C.et al.Nine novel mutation in maturity-onset diabetes of the young (MODY) candidate genes in 22Spanish families[J].J clin Endocrinol Metab,2002,87:2532 ～ 2539
  • 6Winter W E,Nakamura M,House D V.Monogenic diabetes young(MODY1) transcription factor HNF-4Va regulate expression of genes required for glucose transport and metabolism(J).Proc Natl Acad Sci USA,1997,94:13209 ～ 13214
  • 7Stoffers D A,Ferrer J,Clarke W L,et al.Early-onset diabetes mellitus(MODY4) liked to IPF-1[J].Nat Gnet,1997,17:138 ～ 139
  • 8Liza L.llag,Bahman P.Tabaei,William H.Herman,et al:Reduceced pancreatic polypeptide response to typoglycemia and a mylin response to arginine in subjects with a mutation in the HNF-4a/MONDYI gene.Diabetes[J] 2000;49:961 ～ 967
  • 9Yamagata K,Oda N,Kaisaki PJ,et al.Mutations in the hepatocyte nuclear factor 1 alpha gene in maturity-onset diabetes of the young (MODY3)[J].Nature,1996,384:458 ～ 460
  • 10Huang H P,Chu K Nemoz-Gailard E,et al.Neogenesis of beta-cell in adult BETA2/NeoroD-deficient mice[J],Mol Endocrinol,2002,16:541 ～ 551

引证文献4

二级引证文献9

国外医学（内分泌学分册）
2001年 第1期

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