MODY发病的分子和临床病理生理机理
摘要
青年起病的成人型糖尿病（maturity onset diabetes mellitus in youth，MODY）主要是由于胰岛B细胞功能缺陷所致，临床上多表现为不易发生酮症的糖尿病；遵循常染色体显性遗传模式；常在25岁前发病。
出处
《医学新知》
CAS
2006年第5期292-295,共4页
New Medicine
基金
高等学校博士学科点专项科研基金(20030610073)
参考文献21
-
1Njolstad P R,Sovik O,Cuesta-Munoz A,et al.Neonatal diabetes mellitus due to complete glucokinase deficiency.N Engl Med,2001,344:1588
-
2Sturis J,Kurland I J,Byrne M M,et al.Compensation in pancreatic beta-cell function in subjects with glucokinase mutation.Diabetes,1994,43:718
-
3Sreenan S K,Cockburn B N,Baldwin A C,et al.Adaptation to hyperglycemia enhances insulin secretion in glucokinase mutant mice.Diabetes,1998,47:1881
-
4Fajans S S,Brown M B.Administration ofsulfonylureas can increase glucose-induced insulin secretion for decades in patients with maturity-onset diabetes of the young.Diabetes Care,1993,16:1254
-
5Pontoglio M,Sreenan S,Roe M,et al.Defective insulin secretion in hepatocyte nuclear factor 1 deficient mice.J Clin Invest,1998,101:2215
-
6Stoffel M,Duncan S A.The maturity-onset diabetes of the young(MODY 1) transcription factor HNF-4αregulates expression of genes required for glucose transport and metabolism.Proc Natl Acad Sci USA,1997,94:13209
-
7Wang H,Maechler P,Antinozzi P A,et al.Hepatocyte nuclear -4 regulates the expression of pancreatic-cell genes implicated in glucose metabolism and nutrientinduced insulin secretion.J Biol Chem,2000,275:35953
-
8Shih D Q,Dansky H M,Fleisher M,et al.Genotype/phenotype relationships in HNF-4α/MODY1:haploinsufficiency is associated with reduced apolipoprotein (AⅡ),apolipoprotein(CⅢ),lipoprotein(a),and triglyceride levels.Diabetes,2000,49:832
-
9Wright N M,Metzger D l,Borowitz S M,et al.Permanent neonatal diabetes mellitus and pancreatic exocrine insufficiency resulting from congenital pancreatic agenesis.Am J Dis Child,1993,147:607
-
10Clocquet A R,Egan J M,Stoffers D A,et al.Impaired insulin secretion and increased insulin sensitivity in familial maturity-onset diabetes of the young4(insulin promoter factor 1 gene).Diabetes,2000,49:1856
-
1张宗船.胰岛素瘤的诊治进展[J].中国现代普通外科进展,2015,18(7):580-582.
-
2熊红霞.2型糖尿病胰岛B细胞功能缺陷的研究进展[J].中国医学创新,2009,6(19):195-196. 被引量：4
-
3彭姝彬,沈俊,程孟荣,黄青阳.肝细胞核因子4α及1α与糖尿病的关系[J].中国糖尿病杂志,2007,15(4):253-256.
-
4周智广,宁光,贾伟平.中国胰岛素分泌研究组第六次会议纪要：前言[J].中华内分泌代谢杂志,2008,24(4).
-
5曹乐平,朱俊强,石俊.13例胰岛素瘤的诊断与手术治疗[J].中华内分泌外科杂志,2009,3(2):135-136. 被引量：1
-
6崔进贝,冯春芳.胰岛B细胞瘤切除术麻醉体会[J].实用医技杂志,2000,7(6):439-439.
-
7吴怡,赵涵芳.2型糖尿病相关基因的研究进展[J].上海第二医科大学学报,2005,25(6):634-637. 被引量：7
-
8徐常钦,刘景祥.双眼神经纤维瘤合并神经纤维瘤病1例[J].中国实验诊断学,2011,15(10):1783-1783. 被引量：3
-
9王天平,张森,时立新,杨雨星.一个新的肝细胞核因子1α仪基因突变致青少年的成人起病型糖尿病3型家系报道[J].中华糖尿病杂志,2014,6(1):27-31. 被引量：2
-
10刘晓辉,刘太锋,白小军,汪齐.化疗对恶性肿瘤合并2型糖尿病患者胰岛B细胞功能的影响及治疗分析[J].徐州医学院学报,2009,29(12):864-867. 被引量：1
