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Millipore/MAB5554 | Anti-Pax6 Antibody, clone AD1.5/MAB5554/100 µL
Millipore/MAB5554 | Anti-Pax6 Antibody, clone AD1.5/MAB5554/100 µL
市场价:
¥6780.00
美元价:
4068.00
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 | |
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CatalogueNumber | MAB5554 |
BrandFamily | Chemicon® |
TradeName |
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Description | Anti-Pax6Antibody,cloneAD1.5 |
ProductInformation | |
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Format | Ascites |
Control |
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Presentation | Ascitesfluidcontainingnopreservatives. |
StorageandShippingInformation | |
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StorageConditions | Maintainfor1yearat-20°Cfromdateofshipment.Aliquottoavoidrepeatedfreezingandthawing.Formaximumrecoveryofproduct,centrifugetheoriginalvialafterthawingandpriortoremovingthecap. |
BIOLOGicalInformation | |
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Immunogen | RecombinanthumanPax6. |
Clone | AD1.5 |
Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. |
Host | Mouse |
Specificity | RecognizesPax6. |
Isotype | IgG1 |
SpeciesReactivity |
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AntibodyType | MonoclonalAntibody |
EntrezGeneNumber | |
EntrezGeneSummary | Thisgeneencodespairedboxgene6,oneofmanyhumanhomologuesoftheDrosophilamelanogastergeneprd.Inadditiontothehallmarkfeatureofthisgenefamily,aconservedpairedboxdomain,theencodedproteinalsocontainsahomeoboxdomain.BothdomainsareknowntobindDNA,andfunctionasregulatorsofgenetranscription.Thisgeneisexpressedinthedevelopingnervoussystem,andindevelopingeyes.MutationsinthisgeneareknowntocauseaniridiaaswellasPeter"sanomaly,bothoculardiseases. |
GeneSymbol |
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PurificationMethod | Unpurified |
UniProtNumber | |
UniProtSummary | FUNCTION:SwissProt:P26367#Transcriptionfactorwithimportantfunctionsinthedevelopmentoftheeye,nose,centralnervoussystemandpancreas.Requiredforthedifferentiationofpancreaticisletalphacells(Bysimilarity).CompeteswithPAX4inbindingtoacommonelementintheglucagon,insulinandsomatostatinpromoters.RegulatesspecificationoftheventralneuronsubtypesbyestablishingthecorrectProgenitordomains(Bysimilarity).Isoform5aappearstofunctionasamolecularswitchthatspecifiestargetgenes. SIZE:422aminoacids;46683Da SUBCELLULARLOCATION:Nucleus. TISSUESPECIFICITY:Fetaleye,brain,spinalcordandolfactoryepithelium.Isoform5aislessabundantthanthePAX6shorterform. DEVELOPMENTALSTAGE:Expressedinthedevelopingeyeandbrain. DISEASE:SwissProt:P26367#DefectsinPAX6arethecauseofaniridiatypeII(AN2)[MIM:106210].AN2isabilateralpanoculardisordercharacterizedbycompleteorpartialabsenceoftheiris,absenceofthefoveaandmalformationsofthelensandanteriorchamber.Severeage-relatedcornealdegenerationisafrequentcomplicationwhichcontributestoapoorvisualprognostisinaniridia.AboutonethirdofthecasesaresporADIc,andtwothirdsarefamilial,withautosomaldominantinheritanceandhighpenetrance.NearlyonethirdofsporadicANpatientsdevelopWilmstumorinassociationwithgenitourinaryanomaliesandmentalretardation(WAGRsyndrome)asaconsequenceofheterozygous(sub)microscopicdeletionsofchromosome11p13.&DefectsinPAX6areacauseofPetersanomaly[MIM:604229].Petersanomalyconsistsofacentralcornealleukoma,absenceoftheposteriorcornealstromaandDescemetmembrane,andavariabledegreeofirisandlenticularattachmentstothecentralaspectoftheposteriorcornea.&DefectsinPAX6areacauseofectopiapupillae[MIM:129750].Itisacongenitaleyemalformationinwhichthepupilsaredisplacedfromtheirnormalcentralposition.&DefectsinPAX6areacauseoffovealhypoplasia[MIM:136520].Fovealhypoplasiacanbeisolatedorassociatedwithpresenilecataract.Inheritanceisautosomaldominant.&DefectsinPAX6areacauseofautosomaldominantkeratitis[MIM:148190].Itisaneyedisordercharacterizedbycornealopacificationandvascularization,andbyfovealhypoplasia.&DefectsinPAX6areacauseofocularcoloboma[MIM:120200];alsoknownasuveoretinalcolobomaorcolobomaofiris,choroidandretina.Ocularcolobomasareasetofmalformationsresultingfromabnormalmorphogenesisoftheopticcupandstalk,andthefusionofthefetalfissure(opticfissure).Severecolobomatousmalformationsmaycauseasmuchas10%ofthechildhoodblindness.Theclinicalpresentationofocularcolobomaisvariable.Someindividualsmaypresentwithminimaldefectsintheanterioririsleafwithoutotheroculardefects.Morecomplexmalformationscreateacombinationofiris,uveoretinaland/oropticnervedefectswithoutorwithmicrophthalmiaorevenanophthalmia.&DefectsinPAX6areacauseofcolobomaofopticnerve[MIM:120430].&DefectsinPAX6areacauseofbilateralopticnervehypoplasia[MIM:165550];alsoknownasbilateralopticnerveaplasia.Inheritanceisautosomaldominant. SIMILARITY:SwissProt:P26367##Belongstothepairedhomeoboxfamily.&Contains1homeoboxDNA-bindingdomain.&Contains1paireddomain. |
MolecularWeight | 46&48kDa |
PhysicochemicalInformation |
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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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