Dysferlin蛋白抗体实验分析.docx

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1、英文名称Dysferlin中文名称DySfCUin蛋白抗体别名I!T;DYSF;Oyqferlin(Dystrophyassociatedfer1Iikeprotein)(Fer1likeprotein1);DySfCrIinlimbgirdlerrwsculardystrophy2BGUIlcscredrP三1:100-500IHC-F=Iz100500IF=I:100-500Dysforlin3i臼抗体(石蜡切片需做抗原修如notyettestedinotherapplications.opti11iildilutions/conccntrationsshouldbedctcmincdby

2、theenduser.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.Thelyophilizedantibodyisstableatrccntemper

3、atureforatleastonerrrnthandforgrcntcrthanayearhcnkeptat-20tC.WhCnreconstitutedinsterilePH7,l0.OlMPBSordiluentofantibodytheantibodyisstableforatleasttwoweeksat24iC.DySf2rlin蛋臼抗体产品介绍backgrnund:ThoproteinencodedbythisgenebelongstothoferlinfamilyandisaskeletalnuscleproteinfoundassociatedwiththeSarCO)C(T

4、11wIlisinvolvedinIlUSClCcontractionMrldcontainsC2dxnainsthatplayaroleincalciun11ediatedmeubranefusioneventssuggestingthatitnayninvolvedinrftembranoregenerationandrepair.Inaddition,theproteinencodedbythisgenebindsSlVeOIiIr3,MskeletalInJSCICrocmbiancproteinwhichisimportantinthefomtionofcaveolae.Specif

5、icmutationsinthisgenehavebeenShOWntocauseautosonalrecessive1imbgirdle11nsculardystrophytype2B(1.GMD2B)a*veilanMiyoshinyopathy.AlternativesplicingresultsinIiultipletranscriptvarinnl5.providedbyRcfScq,Aug2008.Function:KeycalciumioncensorinvolvedintheCa(2+)-triggeredsynapticvesicle-pIasaiancnbrnncfusion.PlayScchanis11ofbothSkCICItllnuscleandCardianyocytesthatpernitsrapidrescalingofmcabronesdisruptedbynechanicalstress(BysimiIarity).