产品名称：大鼠血管性血友病因子裂解蛋白酶（ADAMTS13/vWF-cp）ELISAKit产品英文：RatvonWillebrandFactorcleavingprotease,ADAMTS-13/vWF-cpELISAKit产品货号：CSB-E11243r产品价格：3200产品规格：96T产品类别：大鼠细胞因子ELISA试剂盒[RatcytokinELISAKit]产品说明：预期应用ELISA法定量测定大鼠血清、血浆或其它相关液体中血管性血友病因子裂解蛋白酶（ADAMTS13/vWF-cp）含量。概述血管性血友病因子裂解蛋白酶一近年来新发现的一种金属蛋白酶,具有降解超大分子血管性血友病因子的作用。实验原理本试剂盒应用双抗体夹心酶标免疫分析法测定标本中ADAMTS13/vWF-cp水平。用纯化的抗体包被微孔板，制成固相抗体，往包被单抗的微孔中依次加入ADAMTS13/vWF-cp抗原、生物素化的抗大鼠ADAMTS13/vWF-cp抗体、HRP标记的亲和素，经过彻底洗涤后用底物TMB显色。TMB在过氧化物酶的催化下转化成蓝色，并在酸的作用下转化成最终的黄色。颜色的深浅和样品中的ADAMTS13/vWF-cp呈正相关。用酶标仪在450nm波长下测定吸光度（OD值），计算样品浓度。IntendeduseThisimmunoassaykitallowsforthespecificmeasurementofratvonWillebrandFactorcleavingprotease,ADAMTS-13/vWF-cpconcentrationsincellculturesupernates,tissuehomogenates,serum,andplasma.IntroductionADAMTS13（ADisintegrinAndMetalloproteinasewithaThromboSpondintype1motif,member13）isazinc-containingmetalloproteaseenzymethatcleavesvonWillebrandfactor（vWf）,alargeproteininvolvedinbloodclotting.ItisalsoknownasvonWillebrandfactor-cleavingprotease（VWFCP）.ItissecretedinbloodanddegradeslargevWfmultimers,decreasingtheiractivity.Genomically,ADAMTS13sharesmanypropertieswiththe19memberADAMTSfamily,allofwhicharecharacterisedbyaproteasedomain（thepartthatperformstheproteinhydrolysis）,anadjacentdisintegrindomainandoneormorethrombospondindomains.ADAMTS13infacthaseightthrombospondindomains.Ithasnohydrophobictransmembranedomain,andhenceitnotanchoredinthecellmembrane.DeficiencyofADAMTS13wasoriginallydiscoveredinUpshaw-Shulmansyndrome,therecurringfamilialformofTTP.BythattimeitwasalreadysuspectedthatTTPoccurredintheautoimmuneformaswell,owingtoitsresponsetoplasmapheresisandcharacterisationofIgGinhibitors.SincethediscoveryofADAMTS13,specificepitopesonitssurfacehavebeenshowntobethetargetofinhibitoryantibodies.Especiallysincethelinkbetweenaorticvalvestenosisandangiodysplasiawasproventobeduetohighshearstress（Heydessyndrome）,ithasbeenacceptedthatincreasedexposureofvWftoADAMTS13duetovariousreasonswouldpredisposetobleedingbycausingincreaseddegradationofvWf.ThisphenomenonischaracterisedbyaformofvonWillebranddisease（type2a）.TestprincipleThisassayemploysthequantitativesandwichenzymeimmunoassaytechnique.AmonoclonalantibodyspecificforADAMTS-13hasbeenpre-coatedontoamicroplate.StandardsandsamplesarepipettedintothewellsandanyADAMTS-13presentisboundbytheimmobilizedantibody.AnbiotinylatedpolyclonalantibodyspecificforADAMTS-13isaddedtothewells.Followingawashtoremoveanyunboundreagent,anenzymecomplexisaddedtothewells.Afterincubationandwashing,asubstratesolutionisaddedtothewellsandcolordevelopsinproportiontotheamountofADAMTS-13boundintheinitialstep.Thecolordevelopmentisstoppedandtheintensityofthecolorismeasured.