Type:MouseIgG
Applications:IF;WB
E=ELISA;FACS;FC=FlowCytometry;FPLC=FastProteinLiquidChromatography;GF=GravityFlow;HPLC=HighPerformanceLiquidChromatography;ICC=Immunocytochemistry;IF=Immunofluorescence;IHC=Immunohistochemistry;IP=Immunoprecipitation;NAC=Non-adherentCellAssays;NB=NeutralizationofBioactivity;SE=SandwichELISA;TPE=TargetedProteinExpression;WB=Westernblotting;;AC=AdherentCellAssays;FM=FluorescentMicsroscopy;;;BSC-CM5=BiacoreSensorChipCM5;BSM=BiosactiveSmallMoleculeorPeptide;CDM=CellDifferentiationMedia;;;;;;HealthandFitness;;;DNAExtraction/Purification;;InvivoLikeAssaysSpeciesReactivity:H;M
B=Bovine;Ca=Cat;Ch=Chicken;D=Dog;EQ=Equine;GP=GuineaPig;H=Human;M=Mouse;P=Porcine;Pr=Primate;R=Rat;Rb=Rabbit;Y=Yeast;Xe=Xenopus;Ze=Zebrafish;;;;NA-NotApplicable;STP=Step-TactinProteins;AllFormat:AffinityPurified-liquid
Immunogen:Humanubiquilin2expressedinandpurifiedfromE.coli
Ubiquilin2,alsoknownasUBQLN2,PLIC2andChap1,isamemberoftheubiquilinproteinfamily,whichregulatethedegradationofcellularproteinsthroughproteasomeorautophage-likepathways.Humanshavefourubiquilingenes,eachencodingaseparateproteinreferredtoasUbiquilin1,2,3and4.AllubiquilinscontainanN-terminalubiquitin-like(UBL)domainandaC-terminalubiquitin-associated(UBA)domain,whilethecentralpartofthemoleculesarehighlyvariable.
Image:HeLacellsstaingwith Ubiquilin2 (green),andcounterstainedwithchickenpolyclonalVimentinantibodyto(red)andDNA(blue).Theantibodyrevealsstrongnuclearlaminastaining,whiletheVimentinantibodyrevealscytoplasmicintermediatefilaments.Protocolondatasheet.
TeepuSiddiqueandhiscollaboratorshaveidentifiedmutationsintheubiquilin2geneleADIngtoproteinpointmutationswhichwereimportantcontributorstoseveralformsofamyotrophiclateralsclerosis(ALS)andFrontotemporallobardegeneration(FTLD).Interestingly,thesemutationsinvolvedalterationsinprolineresiduesinthePXXrepeatregion(P497H,P497S,P506T,P509SandP525S*).Recently,theLeeandTrojanowskigroupinvestigatedC9orf72hexanucleotideexpansionandubiquilin2pathologyinpatientswithALSandFTLDbygeneticanalysisandimmunohistochemistryandfounddistinctubiquilin2pathologyinALSandFTLD-TDPwithC9orf72expansion.***NatureAug21;477(7363):211-52011).**ActaNeuropathol.Jun;123(6):825-392012.
