Description:SpeciesHumanSampleTypeSerumorplasma.ForthequantitativedeterminationofhumanAlpha1-AntitrypsinlevelsinserumorotherBIOLOGicalsamples.Blood,ConnectiveTissueDiseases,LungDiseases,Liver,PathologicProcesses.ProductNameHumanalpha1-Antitrypsin.SampleTypeSerumorplasma.#ofSamples10X96wells.StorageandStABIlity-20°C.RangeofDetection1.4-90ng/mlShelflifeoneyearfromdateofreceipt.AssayConditionThekitperformancehasbeenoptimizedfortheprotocolandmaterialslistedbelowusingstandarddilutionsofhumanAlpha-1-Antitrypsininthe1.4-90ng/mlrange.Theoperatormustdetermineappropriatedilutionsofreagentsforalternativeassayconditions.ELISAassayreactivityissensitivetovariationsinoperator,pipettingandwashingtechniques,incubationtime,temperature,compositionofreagents,andotherexperimentalvariables.Assayoptimizationmayberequiredtogeneratethestandardcurveandfitthesamplesinthespecifieddetectionrange.CountryofOriginUnitedStatesofAmerica.AssayUseForResearchUseOnly(RUO).Notfordiagnosticortherapeuticuseinhumansoranimals.Notforanimalorhumanconsumption.ProtocolsfromPublicationsCitation:1:PottGB,ChanED,DinarelloCA,ShapiroL.Alpha-1-antitrypsinisanendogenousinhibitorofproinflammatorycytokineproductioninwholeblood.JLeukocBiol.2009May;85(5):886-95.Epub2009Feb5.PubMedPMID:19197072;PubMedCentralPMCID:PMC2669404.ExperimentNameELISA.ExperimentBackgroundWholebloodcollectionfromAAT-deficientpatients.AAT-deficientpatientswerediagnosedwithchronicobstructivepulmonarydiseaseandtreatedwithinhaledfluticasone-salmeterol,andeightoftheninepatientsweretreatedwithinhaledalbuterol.Otherprescribedmedicationsincludedfluoxetine,tiotropium,iprotropium,lansoprazole,atorvastatin,alendronate,andfexofenADIne.Humanalpha1-Antitrypsin(AAT)isthemostabundantserineproteaseinhibitorinblood,andAATpossessesanti-inflammatoryactivityinvitroandinvivo.Here,Gregoryet.al.showedthatinvitrostimulationofwholebloodfrompersonswithageneticAATdeficiencyresultedinenhancedcytokineproductioncomparedwithbloodfromhealthysubjects.ExperimentStepsDetermineAATconcentrationsfromAAT-deficientandhealthyvolunteersinheparanizedplasmausingahumanAATquantitativeELISA.FunctionInhibitorofserineproteases.Itsprimarytargetiselastase,butitalsohasamoderateaffinityforplasminandthrombin.Inhibitstrypsin,chymotrypsinandplasminogenactivator.Theaberrantforminhibitsinsulin-inducedNOsynthesisinplatelets,decreasescoagulationtimeandhasproteolyticactivityagainstinsulinandplasmin.SubcellularLocationSecreted.TissueSpecificityPlasma.DomainThereactivecenterloop(RCL)extendsoutfromthebodyoftheproteinanddirectsbindingtothetargetprotease.TheproteasecleavestheserpinatthereactivesitewithintheRCL,establishingacovalentlinkagebetweenthecarboxylgroupoftheserpinreactivesiteandtheserinehydroxyloftheprotease.Theresultinginactiveserpin-proteasecomplexishighlystable.PtmSeveralisomersareobserved,resultingfromthecombinationofdifferentN-linkedglycanstructuresandmatureN-terminus.N-linkedglycanatAsn-107isalternativelydi-antennary,tri-antennaryortetra-antennarywhereasglycanatAsn-70isdi-antennarywithtraceamountsoftri-antennary,andglycanatAsn-271isexclusivelydi-antennary.ThestructureoftheantennasisNeu5Ac(alpha1-6)Gal(beta1-4)GlcNAcattachedtothecorestructureMan(alpha1-6)[Man(alpha1-3)]Man(beta1-4)GlcNAc(beta1-4)GlcNAc.Someantennasarefucosylated,whichformsaLewis-Xdeterminant.ProteolyticprocessingmayyieldthetruncatedformthatrangesfromAsp-30toLys-418.PolymorphismThesequenceshownisthatoftheM1VallelewhichisthemostcommonformofPI(44to49%).Otherfrequentallelesare:M1A20to23%;M210to11%;M314to19%.DiseaseThemajorphysiologicalfunctionofAATistheprotectionofthelowerrespiratorytractagainstproteolyticdestructionbyhumanleukocyteelastase(HLE).AhereditarydeficiencyofAAT,isassociatedwitha20-30foldincreasedriskofdevelopingchronicobstructivepulmonarydisease.DeficiencyofthenormalinhibitorinindividualshomozygousforalleleZorM-Maltoncanresultinthedevelopmentofchronicemphysemaorinfantilelivercirrhosis.VariantPittsburghisthecauseofbleedingdiathesis.MiscellaneousTheaberrantformisfoundintheplasmaofchronicsmokers,andpersistsaftersmokingisceased.Itcanstillbefoundtenyearsaftersmokinghasceased.SimilarityBelongstotheserpinfamily.ProductProtocolToseethisproductusedinapeerreviewedjournal,clickthefollowinglinkα-1-Antitrypsinisanendogenousinhibitorofproinflammatorycytokineproductioninwholeblood.AdditionalInformation:NameHumanAlpha-1-AntitrypsinELISAQuantitationKitRelatedProductNamesAlpha-1proteaseinhibitor,Alpha-1-antiproteinase,Humanalpha1-AntitrypsinELISAKit;Alpha-1proteaseinhibitor,Alpha-1-antiproteinase,AAT,PIHumanalpha1Antitrypsin,SERPINA1,PI,AATELISA,A1ATShortDescriptionHumanalpha1-Antitrypsin,AAT,AATELISANCBIAccNumberNP_000286.3MolecularWeight46737SwissProtNumberP01009IntendedUseResearchUseOnly