Mousemonoclonalantibody
Theantigenisa recombinantfulllengthhumanlaminCexpressedinandpurifiedfromE.Coli.
TheantibodyisProteinGpurifiedfromtissueculturesupernatant.
LaminsAandCarenuclearstructuralproteinsthatarepartoftheintermediatefilamentfamilyandcodedforbythesamegene(LMNA).LaminsAandCarenearlyidenticalexceptfortheircarboxytermini(McKeonetal.,1986).MutationsinthegeneencodinglaminsA/ChavebeenshowntocauseavarietyofdiseasesincludingautosomaldominantEmery-Dreifussmusculardystrophy(Bonneetal.,1995),autosomaldominantlimbgirdlemusculardystrophy(Muchiretal.,2000)andCharcot-Marie-Toothdisordertype2(DeSandre-Giavonnolietal.,2002).
100µl liquidcontaining10mMsodiumazide.
Forlongtermstorage–20°Cisrecommendedinundilutedaliquots.Stableat–20°Cforatleast1year.Avoidfreeze/thawcycles.
BonneG,DiBarlettaMR,VarnousS,BécaneHM,HammoudaEH,MerliniL,MuntoniF,GreenbergCR,GaryF,UrtizbereaJA,DubocD,FardeauM,TonioloD,SchwartzK.(1999)MutationsinthegeneencodinglaminA/CcauseautosomaldominantEmery-Dreifussmusculardystrophy.NatGenet.21(3):285-8
DeSandre-GiovannoliA,ChaouchM,KozlovS,VallatJM,TazirM,KassouriN,SzepetowskiP,HammadoucheT,VandenbergheA,StewartCL,GridD,LévyN.(2002)HomozygousdefectsinLMNA,encodinglaminA/Cnuclear-envelopeproteins,causeautosomalrecessiveaxonalneuropathyinhuman(Charcot-Marie-Toothdisordertype2)andmouse.AmJHumGenet.70(3):726-36.
McKeonFD,KirschnerMW,Caput(1986)Homologiesinbothprimaryandsecondarystructurebetweennuclearenvelopeandintermediatefilamentproteins.Nature319(6053):463-8.
MuchirA,BonneG,vanderKooiAJ,vanMeegenM,BaasF,BolhuisPA,deVisserM,SchwartzK.(2000)IdentificationofmutationsinthegeneencodinglaminsA/Cinautosomaldominantlimbgirdlemusculardystrophywithatrioventricularconductiondisturbances(LGMD1B).HumMolGenet.(9):1453-9.
PhosphoSolutions 由 Drs 于 2001 年创立。Michael D. Browning、John W. Haycock 和 Andrew J. Czernik,他们在 Paul Greengard 博士的实验室共同工作,Paul Greengard 博士因研究蛋白质磷酸化在信号转导中的作用而共同获得 2000 年诺贝尔医学奖在神经系统中。这种专业知识使我们主要关注神经科学领域中感兴趣的蛋白质。我们位于科罗拉多州奥罗拉的 Fitzsimons 生命科学园区,是一支敬业、友好、勤奋的科学家团队,致力于为您提供最优质的抗体和业内客户支持。