Dysferlin蛋白抗体实验分析.docx

英文名称Dysferlin中文名称DySfCUin蛋白抗体别名I!T;DYSF;Oyqferlin(Dystrophyassociatedfer1Iikeprotein)(Fer1likeprotein1);DySfCrIinlimbgirdlerrwsculardystrophy2BGUIlcscredr<xcssivc);Dysferlin1imbgirdlemusculardystrophy2B:Dystrophyassociatedfer1likeI;Dystrophyassociatedfer1likeprotein;Dystrophyassociatedferllike1：Dystrophyassociatedferllikeprotein：Fer1likeprotein1：Ferllikeprotein1：FERlI.1;F1.J00175:F1.J90168;1.GMD2B;1.GMD2B:1.inbgirdlenusculerdystrophy2BGIUtOsCamlrecessive)：1.imbgirdlemusculardystrophy2B;Miyoshimyopathy:MM:DYSI,HUMAN.供应商远疆生物研究领域免疫学通道蛋白抗体来源Rabbit克降类型Polyclonol交叉反应Huxian.Muuse.Rat.Dog.Pig.Cow,Ilnrse,Rabbit.GuineaPig.产品应用WB=I:100-500E1.lSA=I:500I(KK)IP三三1:20-100IHC>P三1:100-500IHC-F=Iz100500IF=I:100-500Dysforlin3i臼抗体(石蜡切片需做抗原修如notyettestedinotherapplications.opti11iildilutions/conccntrationsshouldbedctcmincdbytheenduser.分Tfi237kDa细胞定位细胞膜性状1.yoPhiliZQdor1.iquid浓度lmlnl免及朦K1.HconjugatedsyntheticpeptidederivedfromhunanDysferlin亚型IgG纯化方法affinitypurifiedbyProteinA体存液0.0lMPBS,PH7.4withIOmg/mlBSAand0.l%Sodiunazide保存条件Storeat-200Cforoneyear.Avoidrepeatedfreeze/thawcycles.Thelyophilizedantibodyisstableatrccntemperatureforatleastonerrr>nthandforgrcntcrthanayearhcnkeptat-20tC.WhCnreconstitutedinsterilePH7,l0.OlMPBSordiluentofantibodytheantibodyisstableforatleasttwoweeksat24iC.DySf2rlin蛋臼抗体产品介绍backgrnund:ThoproteinencodedbythisgenebelongstothoferlinfamilyandisaskeletalnuscleproteinfoundassociatedwiththeSarCO)C(T11wIlisinvolvedinIlUSClCcontractionMrldcontainsC2d<xnainsthatplayaroleincalciun11ediatedmeubranefusioneventssuggestingthatitnayninvolvedinrftembranoregenerationandrepair.Inaddition,theproteinencodedbythisgenebindsSlVeOIiIr3,MskeletalInJSCICrocmbiancproteinwhichisimportantinthefomtionofcaveolae.SpecificmutationsinthisgenehavebeenShOWntocauseautosonalrecessive1imbgirdle11nsculardystrophytype2B(1.GMD2B)a*veilanMiyoshinyopathy.AlternativesplicingresultsinIiultipletranscriptvarinnl5.providedbyRcfScq,Aug2008.Function:KeycalciumioncensorinvolvedintheCa(2+)-triggeredsynapticvesicle-pIasaiancnbrnncfusion.PlayS<roleintheSdrcolcinmrepair11>cchanis11ofbothSkCICItllnuscleandCardianyocytesthatpernitsrapidrescalingofmcabronesdisruptedbynechanicalstress(BysimiIarity).