Description | TheLaminproteinsaremembersoftheintermediatefilamentproteinfamilybutarelocatedinsidethenucleusratherthaninthecytoplasm(1).Thelaminsfunctionasskeletalcomponentstightlyassociatedwiththeinnernuclearmembrane.OriginallytheproteinsofthenuclearCytoskeletonwerenamedLaminA,BandC,fromtoptobottomasvisualizedonSDS-PAGEgels.SubsequentlyitwasfoundthatLaminsAandCwerecodedforbyasinglegene(2),whiletheLaminBbandmaycontaintwoproteinsencodedbytwogenesnowcalledLaminB1andLaminB2.LaminAhasamassofabout74kDawhileLaminCis65kDa.TheLaminAproteinincludes98aminoacidsmissingfromLaminC,whileLaminChasaC-terminal6aminoacidpeptidenotpresentinLaminA.ApartfromtheseregionsLaminAandCareidenticalsothatantibodiesraisedagainsteitherproteinarelikelytocrossreactwiththeother,asisthecasewiththismonoclonal.Laminpolymerizationanddepolymerizationisregulatedbyphosphorylationbycyclindependentproteinkinase1(CDK1),thekeycomponentof"maturationpromotingfactor",thecentralregulatorofcelldivision.ActivityofthiskinaseincreasesduringcelldivisionandisresponsIBLeforthebreakdownofthenuclearlamina.MutationsintheLMNAgeneareassociatedwithseveralserioushumandiseases,includingEmery-Dreifussmusculardystrophy,familialpartiallipodystrophy,limbgirdlemusculardystrophy,dilatedcardiomyopathy,Charcot-Marie-Toothdiseasetype2B1,andHutchinson-Gilfordprogeriasyndrome.ThisfamilyofdiseasesbelongtoalargergroupwhichareoftenreferredtoasLaminopathies,thoughsomelaminopathiesareassociatedindefectsinLaminB1,B2oroneorotherofthenumerousnuclearlaminabindingproteins.AtruncatedversionoflaminA,commonlyknownasprogerin,causesHutchinson-Gilfordprogeriasyndrome,aformofprematureaging(3). |
BatchNo. | Seeviallabel |
Unitsize | 100uL |
Antigen | FulllengthrecombinanthumanLaminC |
AntibodyType | Monoclonal |
Isotype | IgG1 |
Producedin | Mouse |
Applications | Immunocytochemistry(ICC),WesternBlotting(WB)andFlowCytometry(~2ugper10^6cells).Adilutionof1:5,000-1:10,000isrecommendedforWB.Adilutionof1:500-1:1000isrecommendedforICC.Theoptimaldilutionshouldbedeterminedbytheenduser. |
Specificity | LaminAandLaminC |
SpeciesAgainst | Human,bovine,porcine,mouseandrat.Itisexpectedthatitwillworkonothermammaltissues. |
Form | LyophilizedfromPBS.Contains5%trehalose. |
Appearance | Whitepowder |
Reconstitution | Reconstituteinsteriledistilledwater.Centrifugetoremoveanyinsolublematerial. |
Storage | Afterreconstitutionoflyophilizedantibody,aliquotandstoreat-20°CforahigherstABIlity.Avoidfreeze-thawcycles. |
ExpiryDate | 12monthsafterpurchase |
GeneralReferences | 1.Fisher,D.Z.,Chaudhary,N.,Blobel,G.CDNAsequencingofnuclearlaminsAandCrevealsprimaryandsecondarystructuralhomologytointermediatefilamentproteins.Proc.Nat.Acad.Sci.83:6450-6454(1986).2.McKeon,F.D.,Kirschner,M.W.,Caput,D.Homologiesinbothprimaryandsecondarystructurebetweennuclearenvelopeandintermediatefilamentproteins.Nature319:463-468(1986).3.Liu,B.andZhou,Z.LaminA/C,laminopathiesandprematureageing.Histol.Histopathol.23:747-763(2006). |
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