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MODY(350001)(maturity-onsetdia2betesoftheyoung,MODY)2(DM),22%5%,,,25,,MODYDM,,2DM,MODY2DMMODY,,B,11990,TanersllFajansMODY1DM,2DM,,,MODY,,MODYHLA,,MODY,,:MODY120q12-q1311,MODY27p,MODY312q2412,MODY413(IPF-1)MODY2(GCK),199612,Yamagata,MODY3,-1(hepatocytenuclearfactor-1,HNF-1),MODY1HNF-4MODY5,,,2DM1MODY1111MODY1HNF-4,HNF-4(ADA),cDNA1992,1996112HNF-4,HNF-4,HNF-1HNF-4(A)(dA),,HNF-4,,5-dATP,dATP,,DNA,,dATP,,,,,,,MODY12113:LindnerT3,,,,,ApoBApoCDM,,,2MODY2GCKMODY2,4MODY,MODY2MODY27p13-15,,12,,GCK,(),GCK,MODY2GCK,,4,,50%55200193,2DM,GCK,DM,,1030,MODY2,GCK,GCK,,,68mmol/L,10mmol/L,,DM,DM,,,ATP,ATPMg2+,,GCK,,3MODY3HNF-1:,MODY3HNF-112q24,MODY3DNA,MODY3DNAHNF-1,,,,BDM,DMMODY2,MengelR5HNF-1,,,,,1DM,,,4MODY4IPF-1:StoffersMODY413(IPF-1)IPF-1STF-1IDX-1PDX-1,,(GLUT2GCK)7,BenIPF-1,,,InoueHIPF-1,,,,,,,:,,5MODY5HNF-1HNF-1TCF2,HNF-1,,HNF-1177(R177X)MODYHorikawa,HNF-1(A263fsinsGG)8,,,Fanconi11HatersleyAT,VehloG,FroguelP,etal:Maturity-onsetdiabetesoftheyoung.BallieresClinPaediabtricsJ1996;4:663680.21LizaL.Ilag,BahmanP.Tabaei,WilliamH.Herman,etal:ReducedpancreaticpolypeptideresponsetotypoglycemiaandamylinresponsetoarginineinsubjuctswithamutationintheHNF-4/MODY1gene.DiabetesJ2000;49:961967.31LindnerT,GragnoliC,FurutaH,etal:Hepaticfunctioninafamilywithanonsensemutations(R154X)inthehepatocytenu2clearfactor-4/MODY1gene.JClinInvestJ1997;100:14001405.41TappyL,DussoixP,IynedjianP,etal:Abnormalregula2tionofhepaticglucoseoutputinmaturity-onsetdiabetesoftheyoungcausedbyaspecificmutationoftheglucokinasegene.Dia2betesJ1997;46:204208.51MengelR,KaisakiPJ,RjasanowskiI,etal:Alowrenalthreshoholdforglucoseindiabeticpatientswithamutationinthehepatocytenuclearfactor-1(HNF-1)gene.DiabMedJ1998;15:816.61Jean-FrancoisSurmely,GUenatE,PhilippeJ,etal:Glu2coseutilizationandproductioninpatientswithmaturity-onsetdi2abetesoftheyoungcausedbyamutationofthehepatocytenuclearfactor-1gene.DiabetesJ1998;47:14591464.71StoffersDA,FerrerJ,ClarkeWL,etal.Early-onsettype-diabetesmellitus(MODY4)linkedtoIPF1.NatGenetJ1997;17:138139.81HorikawaY,IwasakiN,HaraM:Mutationinhepatocytenuclear-1gene(TCF2)associatedwithMODY.NatGeneJ1997;17:384385.(20001009)65200193