tionswithIDDMJ.Diabetes,1998,47:11771184.13BaumH,BrusicV,ChoudhuriK,etal.MHCmolecularmimicryindiabetesJ.NatMed,1995,1:388.14BaumH.MolecularmimicrywithMHC2derivedpeptides:DoesthisunderlieMHCassociationwithautoimmunedisease?J.BiochemSocTrans,1997,25:636647.15HooverML,MartinRT.MolecularmodellingofHLA2DQsug2gestsamechanismofresistanceintype1diabetesJ.ScandJImmunol,1997,45:193202.16SanjeeviCB,DeweeseC,Landin2OllsonM,etal.AnalysisofcriticalresiduesofHLA2DQ6moleculesininsulin2dependentdiabetesmellitusJ.TissueAntigens,1997,50:6165.(:1999201220:1999210212)MODY,(,350001):(MODY)2,,2(HNF)24(GCK)HNF21(IPF)21MODY:MODY;HNF21;HNF24;GCK;IPF21:R587.102:A:1003-5435(2000)01-0042-04MODY2(DM),2DM2%5%,,25,,DMMODY,,,1MODY1DM,2DM,,MODYMODY,HLA,4MODY,:MODY120q12-q1311,MODY27p,MODY312q2412,MODY4:(19762),,13(IPF)2126MODY2(GCKs)1996,Yamagata2,6MODY321(HNF)21;MODY1HNF241MODY1HNF24MODY1(ADA),cDNA1992,199654kb,GC,1211,342451bp,6,14,15,12,2,3(80216000bp),1HNF2443%,5,120bp,HNF24CAAT,TAA22420001201GAA,CAAT,,36sp1,GGGCGGGm,HNF24GC,211%,613%,DNAHNF242,Alu,3Alu,LeuHNF24,HNF21HNF24(A)(dA),,HNF24,,,52dATP,,,DNA,,,dATP,,,,,,MODY17112MODY2GCKMODY2,4MODY,MODY2MODY27p13215,,12,,GCK,(),GCK,,,50%,2DM,GCK,DM,1030,MODY2,GCKGCK,1992VinnetMODYGCK7,GAG279(GLU)TAG()MODY,GCKStoffel7:ThrMetGlyArg,Thr228ATPGly261,MODYHattersley11BXMODY8:Gly299Arg1993Takela5Glu70Lys,Ser131Pro,Ala188Thr,Trp257ArgLys144Glu3(Glu177Lys,Glu256Ala,Lys414Ala),GCKMODY1993Chiu2(PCR2SSCP)113,1212A122DM,GCK9,2DMKazuhiro2092DMGCK,GCKMODY2,MODY2GCK,,12153MODY3HNF21MODY3HNF2112q24,DNA15Pamala,43420001201C(PolyCtrct)C,2904(Pro291fsinsC),,HNF21DNA,Pro291fsinsC,CMODY,TG241,CysGlyTimothy15MODY3HNF218,3(P291fsinsC,P379fsde1CT,A443fsde1CA)5(P129T,R131W,R159W,P519L,T620I)11(73%),ChristianDNAHNF23,23,23,HNF23,23,2324HNF23,23,2324DNA,,Shirou10,Leu254MetMODY3,Leu254HNF21HNF21DNA,MODY3HNF21,,216194MODY4IPF21StoffersMODY413IPF21IPF21STF21IDX21PDX21,,(GLUT2GCK)20,MODY,:HNF21HNF24,HNF21TCF2,HNF21,,,HNF21177(R177X)MODY21Hidekazu,HNF21(A263fsinsGG)22,HNF24:1HattersleyAT,VehloG,FroguelP,etal.Maturity2onsetdia2betesoftheyoungJ.BallClinPaed,1996,4:663680.2YamagataK,FurutaH,OdaN,etal.Mutationsinthehepato2cytenuclearfactor4alphageneinmaturity2onsetdiabetesoftheyoung(MODY1)J.Nature,1996,384:455458.3FroguelP,ZoualiH,VionnetN,etal.Closelinkageofglucoki2naselocusonchromosome7Ptoearly2onsetnon2insulin2de2pendentdiabetesmellitusJ.Nature,1992,356:162164.4VaxillaireM,RouardM,YamagataK,etal.Identificationofninenovelmutationsinthehepatocytenuclearfactor1alphageneassociatedwithmaturity2onsetdiabetesoftheyoung(MODY3)J.HumMolGenet,1997,6:58358615StoffersDA,FerrerJ,ClarkeWL,etal.Early2onsettype2diabetesmellitus(MODY4)linkedtoIPF1J.NatGenet,1997,17:138139.6YamagataK,OdaN,KaisakiPJ,etal.Mutationsinthehepato2cytenuclearfactor1alphageneinmaturity2onsetdiabetesoftheyoung(MODY3)J.Nature,1996,384:458460.7BulmanM,FraylingT,BeardsF,etal.Amissensemutationinthehepatocytenuclearfactor4alphageneinaU.K.pedigreewithmaturity2onsetdiabetesoftheyoung.DiabetologiaJ.1997,40:85986318LindnerT,GragnolicC,FurutaH,etal.Hepaticfunctioninafamilywithanonsensemutations(R154X)inthehepatocytenuclearfactor24/MODY1geneJ.JClinInvest,1997,100:1400140519MaleckiMT.Exclusionofthehepatocytenuclearfactor4asacandidategeneforlate2onsetNIDDMlinkedwithchromosome20qJ.Diabetes,1998,47:970973110SladekFM,DallasYQ,NepomueenoL,etal.MODY1mutationQ268Xinhepatocytenuclearfactor4allowsfordimerizationinsolutionbutcausesabnormalsubcellularlocalizationJ.Diabetes,1998,47:985991111HattersleyAT,BellGI,PolonskyKS,etal.Linkageoftype2diabetestotheglucokinasegeneJ.Lancet,1992,339:13071310112FroguelP,ZoualiH,VionnetN,etal.Familialhyperglycemiaduetomutationsinglucokinase:definitionofasubtypeofdia2betesmellitusJ.NEnglJMed,1993,328:697702113FroguelP,ByrneMM,VehloG,etal.GeneticandmetabolicheterogeneityofmaturityonsetdiabetesoftheyoungJ.Dia2betesRev,1997,5:123130114TappyL,DussoixP,IynedjianP,etal.Abnormalregulationofhepaticglucoseoutputinmaturity2onsetdiabetesoftheyoungcausedbyaspecificmutationoftheglucokinasegeneJ.Di24420001201abetes,1997,46:204208115FraylingTM,BulamnMO,EllardS,etal.Mutationsinthehepatocytenuclearfactor1geneareacommoncauseofmatu2rity2onsetdiabetesoftheyoungintheU.K.J.Diabetes,1997,46:720725116KaisakiPJ,MenzdS,LindnerT,etal.Mutationsinthehepato2cytenuclearfactor1geneinMODYandearly2onsetNIDDM:evidenceforamutationalhospitalinexon4J.Diabetes,1997,45:528535117Jean2FrancoisSurmely,GuenatE,PhilippeT,etal.Glucoseu2tilizationandproductioninpatientswithmaturity2onsetdia2betesoftheyoungcausedbyamutationofthehepatocytenu2clearfactor1geneJ.Diabetes,1998,47:14591464118ByrneMM,SturisJ,MezelS,etal.Alteredinsulinsecretoryre2sponsestoglucoseindiabeticsandnondiabeticsubjectswithmutationsinthediabetessusceptibilitygeneMODY3onchro2mosome12J.Diabetes,1996,45:15031510119BehnPS,ErnestoBM,SusanC,etal.Hepatocytenuclearfactor1codingmutationsareanuncommoncontributortoearly2on2settype2diabetesinAshkenaziJewsJ.Diabetes,1998,47:967969120InoueH,RiggsAC,TanizawaY,etal.Isolation,characteriza2tion,andchromosomal