Anti-desmin/FITC抗体说明书
CatalogNumber:RSF-1026R Quantitysize:0.1ml(2mg/ml;Reconstitutewith0.5mlofsteriledistilledwater.)
Background:filamentsfoundinmusclecells.InadultstriatedmuscletheyformafibrousnetworkconnectingmyofibrilstoeachotherandtotheplasmamembranefromtheperipheryoftheZlinestructures.DefectsinDesminarethecauseofdesminrelatedcardioskeletalmyopathy(CSM)alsoknownasdesminrelatedmyopathy(DRM).CSMischaracterizedbyskeletalmuscleweaknessassociatedwithcardiacconductionblocks,arrhythmias,restrictiveheartfailure,andbyintracytoplasmicaccumulationofdesminreactivedepositsincardiacandskeletalmusclecells.Adesminrelatedmyopathycanhaveadistalonset,itisthenknownashereditarydistalmyopathy(HDM).DefectsinDesminarealsothecauseofdilatedcardiomyopathytype1I(CMD1I).CMD1Iisanautosomalformofdilatedcardiomyopathycharacterizedbyventriculardilatationandimpairedsystolicfunction.Antidesminantibodiesareusefulinidentificationoftumoursofmyogenicorigin.Specificity:
·anti-desmin isarabbitpolyclonalantibody,FITCconjugated·specificfordesminofhuman,mouseandrat
·epitopemappingneartheaminoterminusofdesminofhuman,
mouseratorigin
·human,mouse,rat crossreacted
·affinitychromatographypurification,purity:>95%
·Isotype:IgG
·molwt:25kDa
Application:
·FCM:1:20-100
·Immunohistochemistry:1:20-200
·Immunofluorescence:1:20-200
·Excitationspectrum:490-495nm
·Emissionspectrum:520-530nm
·Optimalworkingdilutionsmustbedeterminedbytheenduser.
Storage:Storeat–20ºCforoneyear.Avoidrepeatedfreeze/thawcycles.Thelyophilizedantibodyisstableatroomtemperatureforatleastonemonthandforgreaterthanayearwhenkeptat-20ºC.WhenreconstitutedinsterilepH7.40.01MPBSordiluentofantibody,theantibodyisstableforatleasttwoweeksat2-4ºC.
ImportantNote:Thisproductassuppliedisintendedforresearchuseonly,notforuseinhuman,therapeuticordiagnosticapplications.
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