密理博/AB9568 |抗神经丝L抗体/AB9568/50µ；L
市场价：
¥5120.00
美元价：
3072.00
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 | AB9568 |
| Replaces | 04-1112 |
| BrandFamily | Chemicon® |
| TradeName |
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| Description | Anti-NeurofilamentLAntibody |
| BackgroundInformation | NeurofilamentsareatypeofintermediatefilamentthatserveasmajorelementsoftheCytoskeletonsupportingtheaxoncytoplasm.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 | |
|---|---|
| Format | Serum |
| Control |
|
| Presentation | Rabbitserum.Containsnopreservative. |
| StorageandShippingInformation | |
|---|---|
| StorageConditions | Stablefor1yearat-20ºCfromdateofreceipt. |
| BIOLOGicalInformation | |
|---|---|
| Immunogen | PurifiedporcineNF-L |
| Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. |
| Host | Rabbit |
| Specificity | RecognizesNeurofilament-Light(NF-L) |
| SpeciesReactivity |
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| AntibodyType | PolyclonalAntibody |
| EntrezGeneNumber | |
| GeneSymbol |
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| PurificationMethod | Unpurified |
| UniProtNumber | |
| UniProtSummary | FUNCTION:SwissProt:P07196#Neurofilamentsusuallycontainthreeintermediatefilamentproteins:L,M,andHwhichareinvolvedinthemaintenanceofneuronalcaliber. SIZE:543aminoacids;61517Da DOMAIN:SwissProt:P07196Theextramassandhighchargedensitythatdistinguishtheneurofilamentproteinsfromallotherintermediatefilamentproteinsareduetothetailpieceextensions.Thisregionmayformachargedscaffoldingstructuresuitableforinteractionwithotherneuronalcomponentsorions. PTM:O-glycosylated(Bysimilarity). DISEASE:SwissProt:P07196#DefectsinNEFLarethecauseofCharcot-Marie-Toothdiseasetype1F(CMT1F)[MIM:607734].CMT1FisaformofCharcot-Marie-Toothdisease,themostcommoninheriteddisorderoftheperipheralnervoussystem.Charcot-Marie-Toothdiseaseisclassifiedintwomaingroupsonthebasisofelectrophysiologicpropertiesandhistopathology:primaryperipheraldemyelinatingneuropathyorCMT1,andprimaryperipheralaxonalneuropathyorCMT2.NeuropathiesoftheCMT1grouparecharacterizedbyseverelyreducednerveconductionvelocities(lessthan38m/sec),segmentaldemyelinationandremyelinationwithonionbulbformationsonnervebiopsy,slowlyprogressivedistalmuscleatrophyandweakness,absentdeeptendonreflexes,andhollowfeet.CMT1Fischaracterizedbyonsetininfancyorchildhood(range1to13years).&DefectsinNEFLarethecauseofCharcot-Marie-Toothdiseasetype2E(CMT2E)[MIM:607684].CMT2EisanautosomaldominantformofCharcot-Marie-Toothdiseasetype2.NeuropathiesoftheCMT2grouparecharacterizedbysignsofaxonalregenerationintheabsenceofobviousmyelinalterations,normalorslightlyreducednerveconductionvelocities,andprogressivedistalmuscleweaknessandatrophy. SIMILARITY:Belongstotheintermediatefilamentfamily. MISCELLANEOUS:NF-Listhemostabundantofthethreeneurofilamentproteinsand,astheothernonepithelialintermediatefilamentproteins,itcanformhomopolymeric10-nmfilaments. |
| MolecularWeight | 68-70kDa |
| 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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