密理博/AB5729 |抗诺金抗体/AB5729/100µ；G
市场价：
¥6580.00
美元价：
3948.00
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 | AB5729 |
| Replaces | MABD184 |
| BrandFamily | Chemicon® |
| TradeName |
|
| Description | Anti-NogginAntibody |
| ProductInformation | |
|---|---|
| Format | AffinityPurified |
| Presentation | Affinitypurifiedimmunoglobulin.Liquid. |
| StorageandShippingInformation | |
|---|---|
| StorageConditions | Maintainat+2-8°Cinundilutedaliquotsforupto6monthsafterdateofreceipt. |
| BIOLOGicalInformation | |
|---|---|
| Immunogen | SyntheticpeptidefrommouseNoggin. |
| Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. |
| Host | Rabbit |
| Specificity | Noggin,mouse. |
| SpeciesReactivity |
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| AntibodyType | PolyclonalAntibody |
| EntrezGeneNumber | |
| EntrezGeneSummary | Thesecretedpolypeptide,encodedbythisgene,bindsandinactivatesmembersofthetransforminggrowthfactor-beta(TGF-beta)superfamilysignalingproteins,suchasbonemorphogeneticprotein-4(BMP4).BydiffusingthroughextracellularmatricesmoreefficientlythanmembersoftheTGF-betasuperfamily,thisproteinmayhaveaprincipalroleincreatingmorphogenicgrADIents.Theproteinappearstohavepleiotropiceffect,bothearlyindevelopmentaswellasinlaterstages.ItwasoriginallyisolatedfromXenopusbasedonitsABIlitytorestorenormaldorsal-ventralbodyaxisinembryosthathadbeenartificiallyventralizedbyUVtreatment.Theresultsofthemouseknockoutoftheorthologsuggestthatitisinvolvedinnumerousdevelopmentalprocesses,suchasneuraltubefusionandjointformation.Recently,severaldominanthumanNOGmutationsinunrelatedfamilieswithproximalsymphalangism(SYM1)andmultiplesynostosessyndrome(SYNS1)wereidentified;bothSYM1andSYNS1havemultiplejointfusionastheirprincipalfeature,andmaptothesameregion(17q22)asthisgene.Allofthesemutationsalteredevolutionarilyconservedaminoacidresidues.TheaminoacidsequenceofthishumangeneishighlyhomologoustothatofXenopus,ratandmouse. |
| GeneSymbol |
|
| UniProtNumber | |
| UniProtSummary | FUNCTION:SwissProt:Q13253#Essentialforcartilagemorphogenesisandjointformation.Inhibitorofbonemorphogeneticproteins(BMP)signalingwhichisrequiredforgrowthandpatterningoftheneuraltubeandsomite(Bysimilarity). SIZE:232aminoacids;25774Da SUBUNIT:Homodimer;disulfide-linked(Bysimilarity). SUBCELLULARLOCATION:Secreted. DISEASE:SwissProt:Q13253#DefectsinNOGarethecauseofsymphalangismproximalsyndrome(SYM1)[MIM:185800].SYM1ischaracterizedbythehereditaryabsenceoftheproximalinterphalangeal(PIP)joints(Cushingsymphalangism).SeverityofPIPjointinvolvementdiminishestowardstheradialside.Distalinterphalangealjointsarelessfrequentlyinvolvedandmetacarpophalangealjointsarerarelyaffectedwhereascarpalbonemalformationandfusionarecommon.Inthelowerextremities,tarsalbonecoalitioniscommon.Conducivehearinglossisseenandisduetofusionofthestapestothepetrouspartofthetemporalbone.&DefectsinNOGarethecauseofmultiplesynostosessyndrome1(SYNS1)[MIM:186500];alsoknownassynostoses,multiple,withbrachydactyly/symphalangism-brachydactylysyndrome.SYNS1ischaracterizedbytubular-shaped(hemicylindrical)nosewithlackofalarflare,otoscleroticdeafness,andmultipleprogressivejointfusionscommencinginthehand.Thejointfusionsareprogressive,commencinginthefifthproximalinterphalangealjointinearlychildhood(oratbirthinsomeindividuals)andprogressinginanulnar-to-radialandproximal-to-distaldirection.Withincreasingage,ankylosisofotherjoints,includingthecervicalvertebrae,hips,andhumeroradialjoints,develop.&DefectsinNOGarethecauseoftarsal-carpalcoalitionsyndrome(TCC)[MIM:186570].TCCisanautosomaldominantdisordercharacterizedbyfusionofthecarpals,tarsalsandphalanges,shortfirstmetacarpalscausingbrachydactyly,andhumeroradialfusion.TCCisallelictoSYM1,anddifferentmutationsinNOGcanresultineitherTCCorSYM1indifferentfamilies.&DefectsinNOGareacauseofstapesankylosiswithbroadthumbandtoes[MIM:184460].Stapesankylosiswithbroadthumbandtoesisacongenitalautosomaldominantdisorderthatincludeshyperopia,ahemicylindricalnose,broadthumbs,greattoes,andotherminorskeletalanomaliesbutlackedcarpalandtarsalfusionandsymphalangism.&DefectsinNOGarethecauseofbrachydactylytypeB2(BDB2)[MIM:611377].BDB2isasubtypeofbrachydactylycharacterizedbyhypoplasia/aplasiaofdistalphalangesincombinationwithdistalsymphalangism,fusionofcarpal/tarsalbones,andpartialcutaneoussyndactyly. SIMILARITY:SwissProt:Q13253##Belongstothenogginfamily. |
| 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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