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Millipore/MAB5552 | Anti-Pax6 Antibody/MAB5552/100 µL
  • Millipore/MAB5552 | Anti-Pax6 Antibody/MAB5552/100 µL

Millipore/MAB5552 | Anti-Pax6 Antibody/MAB5552/100 µL

價(jià)格: ￥4308.00 市場(chǎng)價(jià): 7180.00
貨號(hào): MAB5552
品牌: Millipore
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    • Description
      CatalogueNumberMAB5552
      BrandFamilyChemicon®
      TradeName
      • Chemicon
      DescriptionAnti-Pax6Antibody
      ProductInformation
      FormatAscites
      Control
      • Embryoniceyetissue
      PresentationAscitesfluidcontainingnopreservatives.
      StorageandShippingInformation
      StorageConditionsMaintainfor1yearat-20°Cfromdateofshipment.Aliquottoavoidrepeatedfreezingandthawing.Formaximumrecoveryofproduct,centrifugetheoriginalvialafterthawingandpriortoremovingthecap.
      Applications
      ApplicationDetectPax6usingthisAnti-Pax6AntibodyvalidatedforuseinIH(P)&WB.
      KeyApplications
      • Immunohistochemistry(Paraffin)
      • WesternBlotting
      ApplicationNotesWesternblot.Theantibodyrecognizesthe46and48kDaPax6proteins.

      Immunohistochemistryonparaffinembeddedtissuesections.

      Optimalworkingdilutionsmustbedeterminedbyenduser.
      BIOLOGicalInformation
      ImmunogenRecombinanthumanPax6.
      CloneAD2.38
      ConcentrationPleaserefertotheCertificateofAnalysisforthelot-specificconcentration.
      HostMouse
      SpecificityRecognizesPax6.Theantibodyrecognizesbothproductsofthetwomajoralternativelysplicedforms.
      IsotypeIgG1
      SpeciesReactivity
      • Chicken
      • Human
      • Mouse
      • Rat
      AntibodyTypeMonoclonalAntibody
      EntrezGeneNumber
      EntrezGeneSummaryThisgeneencodespairedboxgene6,oneofmanyhumanhomologuesoftheDrosophilamelanogastergeneprd.Inadditiontothehallmarkfeatureofthisgenefamily,aconservedpairedboxdomain,theencodedproteinalsocontainsahomeoboxdomain.BothdomainsareknowntobindDNA,andfunctionasregulatorsofgenetranscription.Thisgeneisexpressedinthedevelopingnervoussystem,andindevelopingeyes.MutationsinthisgeneareknowntocauseaniridiaaswellasPeter"sanomaly,bothoculardiseases.
      GeneSymbol
      • PAX6
      • WAGR
      • Oculorhombin
      • D11S812E
      • AN2
      • MGDA
      • MGC17209
      • AN
      PurificationMethodUnpurified
      UniProtNumber
      UniProtSummaryFUNCTION:SwissProt:P26367#Transcriptionfactorwithimportantfunctionsinthedevelopmentoftheeye,nose,centralnervoussystemandpancreas.Requiredforthedifferentiationofpancreaticisletalphacells(Bysimilarity).CompeteswithPAX4inbindingtoacommonelementintheglucagon,insulinandsomatostatinpromoters.RegulatesspecificationoftheventralneuronsubtypesbyestablishingthecorrectProgenitordomains(Bysimilarity).Isoform5aappearstofunctionasamolecularswitchthatspecifiestargetgenes.
      SIZE:422aminoacids;46683Da
      SUBCELLULARLOCATION:Nucleus.
      TISSUESPECIFICITY:Fetaleye,brain,spinalcordandolfactoryepithelium.Isoform5aislessabundantthanthePAX6shorterform.
      DEVELOPMENTALSTAGE:Expressedinthedevelopingeyeandbrain.
      DISEASE:SwissProt:P26367#DefectsinPAX6arethecauseofaniridiatypeII(AN2)[MIM:106210].AN2isabilateralpanoculardisordercharacterizedbycompleteorpartialabsenceoftheiris,absenceofthefoveaandmalformationsofthelensandanteriorchamber.Severeage-relatedcornealdegenerationisafrequentcomplicationwhichcontributestoapoorvisualprognostisinaniridia.AboutonethirdofthecasesaresporADIc,andtwothirdsarefamilial,withautosomaldominantinheritanceandhighpenetrance.NearlyonethirdofsporadicANpatientsdevelopWilmstumorinassociationwithgenitourinaryanomaliesandmentalretardation(WAGRsyndrome)asaconsequenceofheterozygous(sub)microscopicdeletionsofchromosome11p13.&DefectsinPAX6areacauseofPetersanomaly[MIM:604229].Petersanomalyconsistsofacentralcornealleukoma,absenceoftheposteriorcornealstromaandDescemetmembrane,andavariabledegreeofirisandlenticularattachmentstothecentralaspectoftheposteriorcornea.&DefectsinPAX6areacauseofectopiapupillae[MIM:129750].Itisacongenitaleyemalformationinwhichthepupilsaredisplacedfromtheirnormalcentralposition.&DefectsinPAX6areacauseoffovealhypoplasia[MIM:136520].Fovealhypoplasiacanbeisolatedorassociatedwithpresenilecataract.Inheritanceisautosomaldominant.&DefectsinPAX6areacauseofautosomaldominantkeratitis[MIM:148190].Itisaneyedisordercharacterizedbycornealopacificationandvascularization,andbyfovealhypoplasia.&DefectsinPAX6areacauseofocularcoloboma[MIM:120200];alsoknownasuveoretinalcolobomaorcolobomaofiris,choroidandretina.Ocularcolobomasareasetofmalformationsresultingfromabnormalmorphogenesisoftheopticcupandstalk,andthefusionofthefetalfissure(opticfissure).Severecolobomatousmalformationsmaycauseasmuchas10%ofthechildhoodblindness.Theclinicalpresentationofocularcolobomaisvariable.Someindividualsmaypresentwithminimaldefectsintheanterioririsleafwithoutotheroculardefects.Morecomplexmalformationscreateacombinationofiris,uveoretinaland/oropticnervedefectswithoutorwithmicrophthalmiaorevenanophthalmia.&DefectsinPAX6areacauseofcolobomaofopticnerve[MIM:120430].&DefectsinPAX6areacauseofbilateralopticnervehypoplasia[MIM:165550];alsoknownasbilateralopticnerveaplasia.Inheritanceisautosomaldominant.
      SIMILARITY:SwissProt:P26367##Belongstothepairedhomeoboxfamily.&Contains1homeoboxDNA-bindingdomain.&Contains1paireddomain.
      MolecularWeight46&48kDa
      PhysicochemicalInformation
      Dimensions
      MaterialsInformation
      MaterialsInformation
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