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Millipore/AB5112 | Anti-Parkin Antibody, a.a. 305-323/AB5112/50 µL

價格: ￥4560.00 市場價: 7600.00

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貨號: AB5112

品牌: Millipore

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Description
CatalogueNumber	AB5112
BrandFamily	Chemicon®
TradeName	Chemicon
Description	Anti-ParkinAntibody,a.a.305-323

ProductInformation
Format	Serum
Presentation	Rabbitserum.Lyophilized.Reconstitutewith50μLofsteriledistilledwater.Centrifugetoremoveinsolublematerial.Containsnopreservative.

StorageandShippingInformation
StorageConditions	Maintainlyophilizedmaterialatat-20°Cto-70°Cforupto12monthsafterdateofreceipt.Afterreconstitutionmaintainat-20°Cto-70°Cinundilutedaliquotsforupto6months.Avoidrepeatedfreeze/thawcycles.Glycerol(ACSgradeorbetter)canbeadded(1:1)forgreaterstABIlity.

Applications
Application	DetectParkinusingthisAnti-ParkinAntibody,a.a.305-323validatedforuseinELISA,WB,IH.
KeyApplications	ELISA WesternBlotting Immunohistochemistry
ApplicationNotes	Immunohistochemistry:1:1000-1:2000 OnesiteELISA Westernblot:1:1000-1:2000(recognizesadoubletat44-52kDaonblotsofhumanbrain) Theimmunogenpeptideisavailable(cat#AG237)forpre-absorbtioncontrols. Optimalworkingdilutionsmustbedeterminedbytheenduser.

BIOLOGicalInformation
Immunogen	A19aminoacidpeptide(RILGEEQYNRYQQYGAEEC)correspondingtoaminoacids305-323ofthehumanparkinmolecule.Aninternalpeptidesequencewaschosentoavoidthepossibilityofcrossreactivitywithubiquitin.
Epitope	a.a.305-323
Host	Rabbit
Specificity	Parkin.Parkinson"sdiseaseisacommonneurodegenerativediseaseiscausedbyslowdeathofneuronsinthesubstantialnigra,abrainregionthatutilizestheneurotransmitterdopamine.Parkin,arecentlydiscoveredgeneencodingalargeprotein,maybeinvolvedinnormalandabnormalproteindegradationincells.RecentevidenceindicatesthatpointmutationsintheParkingeneappeartoberesponsIBLeforthepathogenesisofsomeformsofParkinson"sdisease.
SpeciesReactivity	Human Rat
AntibodyType	PolyclonalAntibody
EntrezGeneNumber	NM_004562.1 NM_013987.1 NM_013988.1
EntrezGeneSummary	Theprecisefunctionofthisgeneisunknown;however,theencodedproteinisacomponentofamultiproteinE3ubiquitinligasecomplexthatmediatesthetargetingofsubstrateproteinsforproteasomaldegradation.MutationsinthisgeneareknowntocauseParkinsondiseaseandautosomalrecessivejuvenileParkinsondisease.Alternativesplicingofthisgeneproducesmultipletranscriptvariantsencodingdistinctisoforms.Additionalsplicevariantsofthisgenehavebeendescribedbutcurrentlylacktranscriptsupport.
GeneSymbol	PARK2 PDJ PRKN LPRS2 AR-JP parkin EC6.3.2.-
UniProtNumber	O60260
UniProtSummary	FUNCTION:SwissProt:O60260#FunctionswithinamultiproteinE3ubiquitinligasecomplex,catalyzingthecovalentattachmentofubiquitinmoietiesontosubstrateproteins.ThesesubstratesincludeSYT11,CCNE1,GPR37,STUB1,a22kDaO-linkedglycosylatedisoformofSNCAIPandSEPT5.Mayplayamoregeneralroleintheubiquitinproteasomalpathwaybyparticipatingintheremovaland/ordetoxificationofabnormallyfoldedordamagedprotein.LossofthisubiquitinligaseactivityappearstobethemechanismunderlyingpathogenesisofPARK2.Mayprotectneuronsagainstalphasynucleintoxicity,proteasomaldysfunction,GPR37accumulation,andkainate-inducedexcitotoxicity.Mayplayaroleincontrollingneurotransmittertraffickingatthepresynapticterminalandincalcium-dependentexocytosis.RegulatescyclinEduringneuronalapoptosis.Mayrepresentatumorsuppressorgene. SIZE:465aminoacids;51641Da SUBUNIT:FormsanE3ubiquitinligasecomplexwithUBE2L3orUBE2L6.PartofaSCF-likecomplex,consistingofPARK2,CUL1andFBXW7.InteractswithSNCAIP.BindstotheC2AandC2BdomainsofSYT11.InteractsandregulatestheturnoverofSEPT5.Partofacomplex,includingSTUB1,HSP70andGPR37.TheamountofSTUB1inthecomplexincreasesduringERstress.STUB1promotesthedissociationofHSP70fromPARK2andGPR37,thusfacilitatingPARK2-mediatedGPR37ubiquitination.HSP70transientlyassociateswithunfoldedGPR37andinhibitstheE3activityofPARK2,whereas,STUB1enhancestheE3activityofPARK2throughpromotionofdissociationofHSP70fromPARK2-GPR37complexes.InteractswithPSMD4andPACRG.InteractswithLRRK2.InteractswithRANBP2.InteractswithSUMO1butnotSUMO2,whichpromotesnuclearlocalizationandautoubiquitination. SUBCELLULARLOCATION:Cytoplasm.Note=Co-localizeswithSTY11inneutrites.Co-localizeswithSNCAIPinbrainstemLewybodies.Nucleus. TISSUESPECIFICITY:Highlyexpressedinthebrainincludingthesubstantianigra.Expressedinheart,testisandskeletalmuscle.Expressionisdown-regulatedorabsentintumorbiopsies,andabsentinthebrainofPARK2patients.Overexpressionprotectsdopamineneuronsfromkainate-mediatedapoptosis. DOMAIN:SwissProt:O60260Theubiquitin-likedomainbindsthePSMD4subunitof26Sproteasomes. PTM:Auto-ubiquitinatesinanE2-dependentmannerleADIngtoitsowndegradation.&S-nitrosylated.TheinhibitionofPARK2ubiquitinE3ligaseactivitybyS-nitrosylationcouldcontributetothedegenerativeprocessinPDbyimpairingtheubiquitinationofPARK2substrates. DISEASE:SwissProt:O60260#DefectsinPARK2areacauseofParkinsondisease(PD)[MIM:168600].PDisacomplex,multifactorialdisorderthattypicallymanifestsaftertheageof50years,althoughearly-onsetcases(before50years)areknown.PDgenerallyarisesasasporadicconditionbutisoccasionallyinheritedasasimplemendeliantrait.AlthoughsporadicandfamilialPDareverysimilar,inheritedformsofthediseaseusuallybeginatearlieragesandareassociatedwithatypicalclinicalfeatures.PDischaracterizedbybradykinesia,restingtremor,muscularrigidityandposturalinstability,aswellasbyaclinicallysignificantresponsetotreatmentwithlevodopa.ThepathologyofPDinvolvesthelossofdopaminergicneuronsinthesubstantianigraandthepresenceofLewybodies(intraneuronalaccumulationsofaggregatedproteins),insurvivingneuronsinvariousareasofthebrain.&DefectsinPARK2arethecauseofautosomalrecessiveearlyonsetParkinsondisease2(PARK2)[MIM:600116];alsoknownasearly-onsetparkinsonismwithdiurnalfluctuation(EPDF)orautosomalrecessivejuvenileParkinsondisease(PDJ).PARK2issymptomaticallydifferentinseveralaspectsfromidiopathicParkinsondisease,althoughclassicsymptomssuchasbradykinesia,rigidityandtremorarepresent.AdditionalclinicalfeaturesincludeearlyDOPA-induceddyskinesia,diurnalfluctuationofthesymptoms,sleepbenefit,dystoniaandhyper-reflexia.PARK2isusuallycharacterizedbyonsetbefore40,withameanageatonsetof23.2years.Pathologically,PARK2patientsshowlossofdopaminergicneuronsinthesubstantianigra,similartothatseeninParkinsondisease;however,Lewybodies(intraneuronalaccumulationsofaggregatedproteins)areabsent.&DefectsinPARK2maybeinvolvedinthedevelopmentand/orprogressionofovariancancer. SIMILARITY:Contains2IBR-typezincfingers.&Contains2RING-typezincfingers.&Contains1ubiquitin-likedomain. MISCELLANEOUS:Theparkinlocus(PRKN),adjacenttothe6qtelomereishyper-recombinableandlieswithinFRA6E,thethirdmostcommonfragilesiteintumortissue.

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Millipore/AB5112 | Anti-Parkin Antibody, a.a. 305-323/AB5112/50 µL

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