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苏州工业园区生物纳米园A4#216
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商品描述
Description | |
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CatalogueNumber | AB5334P |
Replaces | 04-1053 |
BrandFamily | Chemicon® |
TradeName |
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Description | Anti-SynucleinαAntibody |
ProductInformation | |
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Format | AffinityPurified |
Presentation | Affinitypurifiedimmunoglobulin.Lyophilized.Reconstitutewith50μLofsteriledistilledwater.Centrifugetoremoveanyresidue.Glycerol(1:1)canbeaddedforadditionalstABIlity. |
StorageandShippingInformation | |
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StorageConditions | Maintainlyophilizedmaterialat-70°C(dry)forupto12months.Afterreconstitutionmaintainat-20°Cinundilutedaliquotsforupto6months.Avoidrepeatedfreeze/thawcycles. |
BIOLOGicalInformation | |
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Immunogen | Syntheticpeptidecorrespondingaminoacids116-131ofhumanalphasynuclein. |
Host | Sheep |
Specificity | Recognizesalphasynuclein. |
SpeciesReactivity |
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AntibodyType | PolyclonalAntibody |
EntrezGeneNumber | |
EntrezGeneSummary | Alpha-synucleinisamemberofthesynucleinfamily,whichalsoincludesbeta-andgamma-synuclein.Synucleinsareabundantlyexpressedinthebrainandalpha-andbeta-synucleininhibitphospholipaseD2selectively.SNCAmayservetointegratepresynapticsignalingandmembranetrafficking.DefectsinSNCAhavebeenimplicatedinthepathogenesisofParkinsondisease.SNCApeptidesareamajorcomponentofamyloidplaquesinthebrainsofpatientswithAlzheimer"sdisease.TwoalternativelysplicedtranscriptsofSNCAhavebeenidentified.Additionalsplicingmaybepresentbutthefull-lengthnatureofthesevariantshasnotbeendetermined. |
GeneSymbol |
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UniProtNumber | |
UniProtSummary | FUNCTION:SwissProt:P37840#Maybeinvolvedintheregulationofdopaminereleaseandtransport.Solubleprotein,normallylocalizedprimarilyatthepresynapticregionofaxons,whichcanformfilamentousaggregatesthatarethemajornonamyloidcomponentofintracellularinclusionsinseveralneurodegenerativediseases(synucleinopathies).Inducesfibrillizationofmicrotubule-associatedproteintau.Reducesneuronalresponsivenesstovariousapoptoticstimuli,leADIngtoadecreasedcaspase3activation. SIZE:140aminoacids;14460Da SUBUNIT:Solublemonomerwhichcanformfilamentousaggregates.InteractswithUCHL1(Bysimilarity).InteractswithphospholipaseDandhistones. SUBCELLULARLOCATION:Cytoplasm.Membrane.Nucleus.Note=Membrane-boundindopaminergicneurons.Alsofoundinthenucleus. TISSUESPECIFICITY:Expressedprincipallyinbrainbutisalsoexpressedinlowconcentrationsinalltissuesexaminedexceptinliver.Concentratedinpresynapticnerveterminals. DOMAIN:SwissProt:P37840TheNACdomainisinvolvedinthefibrilformation.Themiddleregionformsthecoreofthefilaments.TheC-terminusmayregulateaggregationanddeterminethediameterofthefilaments. PTM:Phosphorylated,predominantlyonserineresidues.PhosphorylationbyCK1appearstooccuronresiduesdistinctfromtheresiduephosphorylatedbyotherkinases.PhosphorylationofSer-129isselectiveandextensiveinsynucleinopathylesions.Invitro,phosphorylationatSer-129promotedinsolublefibrilformation.PhosphorylatedonTyr-125byaPTK2B-dependentpathwayuponosmoticstress.&Hallmarklesionsofneurodegenerativesynucleinopathiescontainalpha-synucleinthatismodifiedbynitrationoftyrosineresiduesandpossIBLybydityrosinecross-linkingtogeneratedstableoligomers.&Ubiquitinated.Thepredominantconjugateisthediubiquitinatedform(Bysimilarity). DISEASE:SwissProt:P37840#DefectsinSNCAareacauseofautosomaldominantParkinsondisease1(PARK1)[MIM:168601,168600].Parkinsondisease(PD)isacomplex,multifactorialdisorderthattypicallymanifestsaftertheageof50years,althoughearly-onsetcases(before50years)areknown.PDgenerallyarisesasasporadicconditionbutisoccasionallyinheritedasasimplemendeliantrait.AlthoughsporadicandfamilialPDareverysimilar,inheritedformsofthediseaseusuallybeginatearlieragesandareassociatedwithatypicalclinicalfeatures.PDischaracterizedbybradykinesia,restingtremor,muscularrigidityandposturalinstability,aswellasbyaclinicallysignificantresponsetotreatmentwithlevodopa.ThepathologyinvolvesthelossofdopaminergicneuronsinthesubstantianigraandthepresenceofLewybodies(intraneuronalaccumulationsofaggregatedproteins),insurvivingneuronsinvariousareasofthebrain.&DefectsinSNCAarethecauseofParkinsondisease4(PARK4)[MIM:605543,168600].&DefectsinSNCAarethecauseofLewybodydementia(DLB)[MIM:127750].DLBisaneurodegenerativedisorderclinicallycharacterizedbydementiaandparkinsonism,oftenwithfluctuatingcognitivefunction,visualhallucinations,falls,syncopalepisodes,andsensitivitytoneurolepticmedication.PresenceofLewybodiesaretheonlyessentialpathologicfeatures.&DepositionoffibrillaramyloidproteinsintraneuronallyasneurofibrillarytanglesischaracteristicofAlzheimerdisease(AD).SNCAisaminorproteinfoundwithinthesedeposits,butamajornonamyloidcomponent.&Brainironaccumulationtype1(NBIA1,alsocalledHallervorden-Spatzsyndrome),arareneuroaxonaldystrophy,ishistologicallycharacterizedbyaxonalspheroids,irondeposition,Lewybody(LB)-likeintraneuronalinclusions,glialinclusionsandneurofibrillarytangles.SNCAisfoundinLB-likeinclusions,glialinclusionsandspheroids. SIMILARITY:Belongstothesynucleinfamily. |
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