Mousemonoclonalantibody
- Size:
- 100µl
- Formulation:
- ProteinGPurified
- Specificity:
- Rat,Mouse,Human,Bovine
- Applications:
- WB1:5,000IF1:1,000
- Species:
- Mouse
- MolecularReference:
- ~64kDaand~74kDa
- CiteThisAntibody:
- PhosphoSolutionsCat#1027-LAM,RRID:AB_2492139
Antigen/Purification:Collapse
Theantigenisa recombinantfulllengthhumanlaminCexpressedinandpurifiedfromE.Coli.
TheantibodyisProteinGpurifiedfromtissueculturesupernatant.
BIOLOGicalSignificance:Collapse
LaminsAandCarenuclearstructuralproteinsthatarepartoftheintermediatefilamentfamilyandcodedforbythesamegene(LMNA).LaminsAandCarenearlyidenticalexceptfortheircarboxytermini(McKeonetal.,1986).MutationsinthegeneencodinglaminsA/ChavebeenshowntocauseavarietyofdiseasesincludingautosomaldominantEmery-Dreifussmusculardystrophy(Bonneetal.,1995),autosomaldominantlimbgirdlemusculardystrophy(Muchiretal.,2000)andCharcot-Marie-Toothdisordertype2(DeSandre-Giavonnolietal.,2002).
Storage
100µl liquidcontaining10mMsodiumazide.
Forlongtermstorage–20°Cisrecommendedinundilutedaliquots.Stableat–20°Cforatleast1year.Avoidfreeze/thawcycles.
GeneralReferences
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.
