Millipore/CBL275 |抗神经粘附分子L1抗体，克隆UJ127.11/CBL275/100µ；G
市场价：
¥0.00
美元价：
0.00
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 | CBL275 |
| BrandFamily | Chemicon® |
| TradeName |
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| Description | Anti-NeuralAdhesionMoleculeL1Antibody,cloneUJ127.11 |
| AlternateNames |
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| BackgroundInformation | Familiesofadhesionmoleculeswhichsharecommoncarbohydratedomainsdoexist,despitethestructuralandfunctionaldiversityoftheseglycoproteins.TheseincludetheCa2+-independentneuraladhesionmolecules:N-CAM,myelinassociatedglycoprotein(MAG)andL1.L1isinvolvedinneuron-neuronadhesion,neuritefasciculation,outgrowthofneurites,cerebellargranulecellmigration,neuriteoutgrowthonSchwanncellsandinteractionsamongepithelialcellsofintestinalcrypts |
| ProductInformation | |
|---|---|
| Format | Purified |
| Presentation | Themonoclonalispresentedataconcentrationof100μg/1mlinphosphatebufferedsalinecontaining10mMsodiumazideand1mg/mlbovineserumalbumin.Werecommendthateachlaboratorydetermineanoptimumworkingtitreforuseinitsparticularapplication. |
| StorageandShippingInformation | |
|---|---|
| StorageConditions | Forusewithin1monthofpurchasestoreat+4°C,forlongtermstoragealiquotantibodyintosmallvolumesandstoreat-20°C. |
| BIOLOGicalInformation | |
|---|---|
| Immunogen | Homogenateof16weekhumanfoetalbraincells. |
| Clone | UJ127.11 |
| Host | Mouse |
| Specificity | Theantibodyreactswiththe220-240kDaglycoproteinL1moleculewhichisexpressedinneuraltissues,neuroblastomaandmedulloblastoma.L1NCAMexpressionalsooccursoncellsofmyelomonocyticandlymphoidorigin. FUSIONPARTNER:P3-X63-Ag8.653myelomacellline. |
| Isotype | IgG1 |
| SpeciesReactivity |
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| AntibodyType | MonoclonalAntibody |
| GeneSymbol |
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| UniProtNumber | |
| UniProtSummary | Function:Celladhesionmoleculewithanimportantroleinthedevelopmentofthenervoussystem.Involvedinneuron-neuronadhesion,neuritefasciculation,outgrowthofneurites,etc.Bindstoaxoninonneurons. Subcellularlocation:Cellmembrane;Single-passtypeImembraneprotein. Involvementindisease:DefectsinL1CAMarethecauseofhydrocephalusduetostenosisoftheaqueductofSylvius(HSAS)[MIM:307000].Hydrocephalusisaconditioninwhichabnormalaccumulationofcerebrospinalfluidinthebraincausesincreasedintracranialpressureinsidetheskull.Thisisusuallyduetoblockageofcerebrospinalfluidoutflowinthebrainventriclesorinthesubarachnoidspaceatthebaseofthebrain.Inchildrenistypicallycharacterizedbyenlargementofthehead,prominenceoftheforehead,brainatrophy,mentaldeterioration,andconvulsions.Inadultsthesyndromeincludesincontinence,imbalance,anddementia.HSASischaracterizedbymentalretardationandenlargedbrainventricles. DefectsinL1CAMarethecauseofmentalretardation-aphasia-shufflinggait-adductedthumbssyndrome(MASA)[MIM:303350];alsoknownascorpuscallosumhypoplasia,psychomotorretardation,adductedthumbs,spasticparaparesis,andhydrocephalusorCRASHsyndrome.MASAisanX-linkedrecessivesyndromewithahighlyvariableclinicalspectrum.Mainclinicalfeaturesincludespasticityandhyperreflexiaoflowerlimbs,shufflinggait,mentalretardation,aphasiaandadductedthumbs.Thefeaturesofspasticityhavebeenreferredtoascomplicatedspasticparaplegiatype1(SPG1).Somepatientsmanifestcorpuscallosumhypoplasiaandhydrocephalus.Inter-andintrafamilialvariABIlityisverywide,suchthatpatientswithhydrocephalus,MASA,SPG1,andagenesisofcorpuscallosumcanbepresentwithinthesamefamily. DefectsinL1CAMarethecauseofspasticparaplegiaX-linkedtype1(SPG1)[MIM:303350].Spasticparaplegiaisadegenerativespinalcorddisordercharacterizedbyaslow,gradual,progressiveweaknessandspasticityofthelowerlimbs. DefectsinL1CAMmaycontributetoHirschsprungdisease(HSCR)[MIM:142623].ItmaydosobymodifyingtheeffectsofaHirschsprungdisease-associatedgenetocauseintestinalaganglionosis. DefectsinL1CAMareacauseofpartialagenesisofthecorpuscallosum[MIM:304100];aX-linkeddisorder. Sequencesimilarities:Belongstotheimmunoglobulinsuperfamily.L1/neurofascin/NgCAMfamily. Contains5fibronectintype-IIIdomains. Contains6Ig-likeC2-type(immunoglobulin-like)domains. |
| PhysicochemicalInformation |
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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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