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Millipore/MAB2265 | Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1/MAB2265/100 µg
Millipore/MAB2265 | Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1/MAB2265/100 µg
市场价：
¥6360.00
美元价：
3816.00
产品分类：
功能性抗体
公司分类：
Functional_antibody
联系Q Q：
3392242852
电话号码：
4000-520-616
电子邮箱：
info@ebiomall.com
商品介绍
| Description | |
|---|---|
| CatalogueNumber | MAB2265 |
| Description | Anti-PeripheralMyelinProtein22(PMP22)Antibody,cloneCF1 |
| AlternateNames |
|
| BackgroundInformation | Peripheralmyelinprotein22(PMP22),a160aminoacidglycoprotein,belongstotheclaudinfamilyofproteins.PMP22isthoughttohaveacriticalroleinexternalmesaxonformationduringdevelopmentaswellaspotentialinvolvementintheprogressionofaxonmyelination.ThisglycoproteinhasfourhydrophobicdomainsafeaturewhichgivesrisetoanotherpotentialroleforPMP22inthestructureofperipheralnervemyelin.PMP22hasbeenobservedincompactmyelinfoundwithintheperipheralnerveofhumanadult,andhasbeennotedashavingadistributionsimilartothatofmyelinProteinzero(PO).DefectsinPMP-22expressionarecausaltoseveralhereditarydemyelinatingneuropathiesincluding;inflammatorydemyelinatingpolyneuropathy(IDP),hereditaryneuropathywithliABIlitytopressurepalsies(HNPP),Charcot-Marie-ToothdiseaseType1A(CMT1A)andtype1E(CMT1E),andDejerine-Sottassyndrome(DSS). |
| ProductInformation | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | PurifiedmousemonoclonalIgG1κinbuffercontaining0.1MTris-Glycine(pH7.4),150mMNaClwith0.05%sodiumazide. |
| StorageandShippingInformation | |
|---|---|
| StorageConditions | Stablefor1yearat2-8°Cfromdateofreceipt. |
| Applications | |
|---|---|
| Application | Anti-PeripheralMyelinProtein22(PMP22)Antibody,cloneCF1isanantibodyagainstPeripheralMyelinProtein22(PMP22)foruseinIH. |
| KeyApplications |
|
| BIOLOGicalInformation | |
|---|---|
| Immunogen | HumanPMP22CDNAboostedwith13-merpeptideofthesecondextracellulardomainofPMP22 |
| Epitope | Unknown |
| Clone | CF1 |
| Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. |
| Host | Mouse |
| Specificity | Thisantibodyrecognizesperipheralmyelinprotein22(PMP22) |
| Isotype | IgG1κ |
| SpeciesReactivity |
|
| AntibodyType | MonoclonalAntibody |
| EntrezGeneNumber | |
| EntrezGeneSummary | Thisgeneencodesanintegralmembraneproteinthatisamajorcomponentofmyelinintheperipheralnervoussystem.VariousmutationsofthisgenearecausesofCharcot-Marie-ToothdiseaseTypeIA,Dejerine-Sottassyndrome,andhereditaryneuropathywithliabilitytopressurepalsies.Alternativesplicingofthisgeneresultsinthreetranscriptvariantsthatencodethesameprotein.[providedbyRefSeq]. |
| GeneSymbol |
|
| PurificationMethod | ProteinG |
| UniProtNumber | |
| UniProtSummary | FUNCTION:Mightbeinvolvedingrowthregulation,andinmyelinizationintheperipheralnervoussystem. SUBCELLULARLOCATION:Membrane;Multi-passmembraneprotein. INVOLVEMENTINDISEASE:DefectsinPMP22arethecauseofCharcot-Marie-Toothdiseasetype1A(CMT1A)[MIM:118220];alsoknownashereditarymotorandsensoryneuropathyIA.CMT1AisaformofCharcot-Marie-Toothdisease,themostcommoninheriteddisorderoftheperipheralnervoussystem.Charcot-Marie-Toothdiseaseisclassifiedintwomaingroupsonthebasisofelectrophysiologicpropertiesandhistopathology:primaryperipheraldemyelinatingneuropathyorCMT1,andprimaryperipheralaxonalneuropathyorCMT2.NeuropathiesoftheCMT1grouparecharacterizedbyseverelyreducednerveconductionvelocities(lessthan38m/sec),segmentaldemyelinationandremyelinationwithonionbulbformationsonnervebiopsy,slowlyprogressivedistalmuscleatrophyandweakness,absentdeeptendonreflexes,andhollowfeet.CMT1Ainheritanceisautosomaldominant. DefectsinPMP22areacauseofDejerine-Sottassyndrome(DSS)[MIM:145900];alsoknownasDejerine-Sottasneuropathy(DSN)orhereditarymotorandsensoryneuropathyIII(HMSN3).DSSisaseveredegeneratingneuropathyofthedemyelinatingCharcot-Marie-Toothdiseasecategory,withonsetbyage2years.DSSischaracterizedbymotorandsensoryneuropathywithveryslownerveconductionvelocities,increasedcerebrospinalfluidproteinconcentrations,hypertrophicnervechanges,delayedageofwalkingaswellasareflexia.TherearebothautosomaldominantandautosomalrecessiveformsofDejerine-Sottassyndrome.Ref.10Ref.11Ref.14Ref.15Ref.18Ref.19Ref.20Ref.21Ref.24Ref.25Ref.26Ref.28Ref.31Ref.33Ref.37Ref.41 DefectsinPMP22areacauseofhereditaryneuropathywithliabilitytopressurepalsies(HNPP)[MIM:162500];anautosomaldominantdisordercharacterizedbytransientepisodesofdecreasedperceptionorperipheralnervepalsiesafterslighttraction,compressionorminortraumas.Ref.29Ref.44Ref.45 DefectsinPMP22arethecauseofCharcot-Marie-Toothdiseasetype1E(CMT1E)[MIM:118300];alsoknownasCharcot-Marie-Toothdiseaseanddeafnessautosomaldominant.CMT1EisanautosomaldominantformofCharcot-Marie-Toothdiseasecharacterizedbytheassociationofsensorineuralhearinglosswithperipheraldemyelinatingneuropathy. DefectsinPMP22maybeacauseofinflammatorydemyelinatingpolyneuropathy(IDP)[MIM:139393].IDPisaputativeautoimmunedisorderpresentinginanacute(AIDP)orchronicform(CIDP).TheacuteformisalsoknownasGuillain-Barresyndrome. SEQUENCESIMILARITIES:BelongstothePMP-22/EMP/MP20family. |
| MolecularWeight | 18kDacalculated |
| PhysicochemicalInformation |
|---|
| Dimensions |
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| MaterialsInformation |
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| MaterialsInformation |
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品牌介绍
密理博(Millipore)公司成立于1954年，总部位于美国麻省，在全世界设有47个办事处，为100多个国家提供产品和技术服务。目前全球雇员超过5800人，在美国、法国和日本等国家拥有7家大型生产工厂，主要生产过滤膜及膜过滤产品。20世纪80年代，密理博公司进入中国市场。先后在香港、北京、上海、广州及成都设立了办事机构，并于2000年4月在上海浦东外高桥保税区建立了密理博（上海）贸易有限公司。为了更好地满足中国用户的需求，密理博中国主页于2006年11月向广大用户开放，介绍密理博中国有限公司的最新动态，力求为用户打造专业的产品与服务信息交流平台。
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