Kitsize | 12x8 |
Method | ELISA |
Incubationtime | 3x1h,1x30min |
Standardrange | 0-12U/ml |
Specimen/Volumes | 10µlserum |
Substrate/isotope | PNPP405nm |
RegulatoryStatus: | EU:CE |
SEROnegativemyastheniagravisisnolongerseronegative
Myastheniagravis(MG)iscausedbythefailureofneuromuscularsignaltransmissionandinthemajorityofpatientsthisiscausedbythepresenceofautoantibodiestotheacetylcholinereceptor(ARAbpositiveMGpatients).In10-20%ofthepatientswithgeneralizedMG,andinupto50%ofthepatientswithocularMGtheseautoantibodiescannotbedetected.InthesecasesthediseaseiscommonlyreferredtoasseronegativeMG(SNMG).ForalongtimetheseseronegativepatientsposedaseriouschallengeforadequatediagnosisofMG.
Recentstudiesshowedthepresenceofantibodiestothemuscle-specificreceptortyrosinekinase(MuSK)inseraofpatientswithgeneralizedseronegativeMG(inabout50%ofputativeseronegativeMGpatients).MuSKispartofanagrinreceptorcomplexandmediatestheagrin-inducedclusteringofacetylcholinereceptors.MuSKMGpatientsaremostlyfemale,haveprominentcranialandbulbarinvolvement,and(whencomparedtoARAbpositiveMGpatients)theonsetofthediseasetendstobeearlier(thirdorfourthdecade).ThemeasurementofMuSKantibodieswillsubstantiallyaiddiagnosisandclinicalmanagementofMG.
MuSK-AbELISAissuperiortoonlycommerciallyavailableRIA
DataonthequalitativecomparisonofthemeasurementsofMuSKautoantibodiesin253serumsamplesshowthesuperiorityoftheIBLMuSK-AbELISAcomparedtotheonlycommerciallyavailableMuSK-AbRIA.
MuSK-AbRIA | |||
positiv | negativ | ||
IBLInternationalMuSK-AbELISA | positiv | 92 | 0 |
negativ | 4 | 157 |
ExcerptfromtheInstructionsforUse
Myastheniagravis(MG)iscausedbythefailureofneuromusculartransmissionmediatedbyautoantibodies.About80to90%ofpatientswithgeneralizedMGhaveautoantibodiesagainstthemusclenicotinicacetylcholinereceptors.Theseantibodiescauselossofacetylcholinereceptornumbersandfunction,andleadtofailureofneuromusculartransmissionwithmuscleweakness.In10–20%ofpatientswithgeneralizedmyastheniagravisandinupto50%ofpatientswithocularmyastheniagravistherearenodetectableantibodiestotheacetylcholinereceptor.Insuchcasesthediseaseiscommonlyreferredtoasseronegativemyastheniagravis.Seronegativemyastheniagravishasbeenrecognizedasanantibodymediateddisease.Antibodiestomuscle-specificreceptortyrosinekinase(MuSK)weredemonstratedintheseraofpatientswithgeneralizedseronegativemyastheniagravis(inapproximately40%ofseronegativeMGpatients).MuSKispartofanagrinreceptorcomplexandmediatestheagrin-inducedclusteringofacetylcholinereceptorsduringsynapseformation,andisalsoexpressedatthematureneuromuscularjunction(NMJ).MuSKMGpatientsarepredominantlyfemale,haveprominentcranialandbulbarinvolvement,andtendtohaveahigherrateofcrisesthanthosewithotherformsofMG.Diseaseonsettendstobeearlier,withmostpatientspresentingbythethirdorfourthdecade.ThemeasurementofMuSKantibodieswillsubstantiallyaiddiagnosisandclinicalmanagementofMyastheniagravis.
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