Type
Recombinant
Description
C1Inhibitorisamemberoftheserpinfamilyofstructurallyrelatedproteinsandistheprimaryregulatoroftheimmunecomplementsystem.C1Inhibitorisaproteaseinhibitorthatfunctionstoinhibitthecomplementsysteminordertopreventover-activationorspontaneousactivation.InhibitionisachievedbybindingtoandirreversIBLyinhibitingtheC1randC1sproteasesoftheC1complex,whichhastheeffectofshuttingdownallsubsequentdownstreameventsinthecomplementactivationcascade.C1inhibitorcanalsoinhibitvariousotherproteases,includingKallikrein,FactorXIaandFactorXIIa.DeficienciesinC1inhibitoraretheprimarycauseofhereditaryangioedema(HAE,hereditaryangioneuroticedema),adiseasecharacterizedbyedemaintherespiratoryandgastrointestinaltracts.Incertainclinicalsituations,thedirectadmiNISTrationofC1inhibitorcanbeusedtotreatHAEandcertainotherconditions.RecombinantHumanC1Inhibitorisahighlyglycosylatedglycoproteincontaining445 aminoacidresidues(49.4kDa),correspondingtoaminoacids56 –500 oftheC1inhibitorprecursorandisfullyfunctionalinitsABIlitytoinhibittheC1complex.GlycosylatedC1Inhibitormigratesatanapparentmolecularweightofapproximately80–90kDabySDSPAGEanalysisunderreducingconditions.
AminoAcidSequence
VEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA
Source
ChineseHamsterOvaryCells(CHO)
Purity
95%
BIOLOGicalActivity
MeasuredbyitsabilitytoinhibitrecombinanthumancomplementcomponentC1acleavageofacolorimetricpeptidesubstrate,NCarbobenzyloxy-Lys-ThioBenzylester(Z-K-SBzl).TheexpectedIC50is≤2.6 nM.
Endotoxin
Endotoxinlevelis<0.1 ng/μgofprotein(<1EU/μg).
Reconstitution
Centrifugethevialpriortoopening.Reconstituteinwatertoaconcentrationof0.1–1.0 mg/ml.Donotvortex.Forextendedstorage,itisrecommendedtofurtherdiluteinabuffercontainingacarrierprotein(example0.1%BSA)andstoreinworkingaliquotsat –20°Cto –80°C.
Storage/Expiration
–20°C
