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Millipore/MAB5256X | Anti-Neurofilament H Antibody, clone NE14, Alexa Fluor? 488 Conjugated/MAB5256X/40 µg
  • Millipore/MAB5256X | Anti-Neurofilament H Antibody, clone NE14, Alexa Fluor? 488 Conjugated/MAB5256X/40 µg

Millipore/MAB5256X | Anti-Neurofilament H Antibody, clone NE14, Alexa Fluor? 488 Conjugated/MAB5256X/40 µg

價格: ￥4812.00 市場價: 8020.00
貨號: MAB5256X
品牌: Millipore
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    • Description
      CatalogueNumberMAB5256X
      DescriptionAnti-NeurofilamentHAntibody,cloneNE14,AlexaFluor?488Conjugated
      AlternateNames
      • 200kDaneurofilamentprotein
      • NeurofilamenttripletHprotein
      • neurofilament,heavypolypeptide
      • neurofilament,heavypolypeptide200kDa
      BackgroundInformationNeurofilamentsareatypeofintermediatefilamentthatserveasmajorelementsoftheCytoskeletonsupportingtheaxoncytoplasm.Theyarethemostabundantfibrillarcomponentsoftheaxon,beingonaverage3-10timesmorefrequentthanaxonalmicrotubules.Neurofilaments(10nmindia.)arebuiltfromthreeintertwinedprotofibrilswhicharethemselvescomposedoftwotetramericprotofilamentcomplexesofmonomericproteins.Theneurofilamenttripletproteins(68/70,160,and200kDa)occurinboththecentralandperipheralnervoussystemandareusuallyneuronspecific.The68/70kDaNF-Lproteincanself-assembleintoafilamentousstructure;howeverthe160kDaNF-Mand200kDaNF-Hproteinsrequirethepresenceofthe68/70kDaNF-Lproteintoco-assemble.Neuromas,ganglioneuromas,gangliogliomas,ganglioneuroblastomasandneuroblastomasstainpositivelyforneurofilaments.Althoughtypicallyrestrictedtoneurons,neurofilamentshavebeendetectedinparagangliomasandadrenalandextra-adrenalpheochromocytomas.Carcinoids,neuroendocrinecarcinomasoftheskin,andoatcellcarcinomasofthelungalsoexpressneurofilaments.
      ProductInformation
      FormatAlexaFluor?488
      Control
      • RatCortexCells.
      PresentationPurifiedimmunoglobulinin0.02MPhosphatebuffer,0.25MNaClcontaining0.1%sodiumazide.
      StorageandShippingInformation
      StorageConditionsStablefor6monthsat2-8oCfromdateofreceipt.
      Applications
      ApplicationAnti-NeurofilamentHAntibody,cloneNE14,AlexaFluor488ConjugatedisanantibodyagainstNeurofilamentHforuseinIH&IC.
      KeyApplications
      • Immunohistochemistry
      • Immunocytochemistry
      ApplicationNotesImmunohistochemistry:
      5-10μg/mLonfrozenbraintissue.Optimalworkingdilutionsmustbedeterminedbyenduser.
      BIOLOGicalInformation
      ImmunogenPurifiedneurofilamentpolypeptides(Debusetal.,1983).
      EpitopeUnknown
      CloneNE14
      ConcentrationPleaserefertotheCertificateofAnalysisforthelot-specificconcentration.
      HostMouse
      SpecificityThisantibodyrecognizesNeurofilamentH.
      IsotypeIgG1
      SpeciesReactivity
      • Rat
      • Human
      • Pig
      SpeciesReactivityNoteHuman,PorcineandRat.Reactivitywithotherspecieshasnotbeentested.
      AntibodyTypeMonoclonalAntibody
      EntrezGeneNumber
      EntrezGeneSummaryNeurofilamentsaretypeIVintermediatefilamentheteropolymerscomposedoflight,medium,andheavychains.Neurofilamentscomprisetheaxoskeletonandfunctionallymaintainneuronalcaliber.Theymayalsoplayaroleinintracellulartransporttoaxonsanddendrites.Thisgeneencodestheheavyneurofilamentprotein.ThisproteiniscommonlyusedasabioMarkerofneuronaldamageandsusceptibilitytoamyotrophiclateralsclerosis(ALS)hasbeenassociatedwithmutationsinthisgene.[providedbyRefSeq].
      GeneSymbol
      • NEFH
      • NF-H
      • NFH
      PurificationMethodProteinGPurified
      UniProtNumber
      UniProtSummaryFUNCTION:Neurofilamentsusuallycontainthreeintermediatefilamentproteins:L,M,andHwhichareinvolvedinthemaintenanceofneuronalcaliber.NF-HhasanimportantfunctioninmatureaxonsthatisnotsubservedbythetwosmallerNFproteins.

      PTM:ThereareanumberofrepeatsofthetripeptideK-S-P,NFHisphosphorylatedonanumberoftheserinesinthismotif.ItisthoughtthatphosphorylationofNFHresultsintheformationofinterfilamentcrossbridgesthatareimportantinthemaintenanceofaxonalcaliber.

      Phosphorylationseemstoplayamajorroleinthefunctioningofthelargerneurofilamentpolypeptides(NF-MandNF-H),thelevelsofphosphorylationbeingaltereddevelopmentallyandcoincidentwithachangeintheneurofilamentfunction.

      POLYMORPHISM:Thenumberofrepeatsisshowntovarybetween29and30.

      DISEASE:DefectsinNEFHareacauseofsusceptibilitytoamyotrophiclateralsclerosis(ALS)[MIM:105400].ALSisaneurodegenerativedisorderaffectingupperandlowermotorneurons,andresultinginfatalparalysis.Sensoryabnormalitiesareabsent.Deathusuallyoccurswithin2to5years.Theetiologyislikelytobemultifactorial,involvingbothgeneticandenvironmentalfactors.Ref.8

      SIMILARITY:Belongstotheintermediatefilamentfamily.

      MolecularWeight~200kDa
      PhysicochemicalInformation
      Dimensions
      MaterialsInformation
      MaterialsInformation
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