请使用支持JavaScript的浏览器!
主营:抗体类、生化类试剂、ELISA试剂盒、组织染色细胞可视化试剂、针对神经科学和细胞疾病研究产品和纯化蛋白等
℡ 4000-520-616
℡ 4000-520-616
Biosensis/Mouse monoclonal to human Deafness dystonia protein 1 [2F11]: IgG/M-831-100/100 µg
产品编号:M-831-100
市  场 价:¥6540.00
场      地:美国(厂家直采)
产品分类: 抗体类>一抗>单克隆抗体>
联系QQ:1570468124
电话号码:4000-520-616
邮      箱: info@ebiomall.com
美  元  价:$327.00
品      牌: Biosensis
公      司:Biosensis
公司分类:
Biosensis/Mouse monoclonal to human Deafness dystonia protein 1 [2F11]: IgG/M-831-100/100 µg
商品介绍
Description
TIMM8Aisamitochondrialintermembranechaperonethatisinvolvedintheimportandinsertionofsomemulti-passtransmembraneproteinsintothemitochondrialinnermembrane.Italsoactsasachaperone-likeproteinthatprotectsthehydrophobicprecursorsfromaggregationandguidethemthroughthemitochondrialintermembranespace.TIMM8Aisprobablynecessaryfornormalneurologicdevelopment.TIMM8AisaheterohexamercomposedofthreeTIMM8AandthreeTIMM13subunits,namedsoluble70KDacomplex.ItassociateswiththeTIMM22complex.Itislocatedatthemitochondrioninnermembraneandishighlyexpressedinfetalandadultbrain,followedbyfetallung,liverandkidney.DefectsinTIMM8AcauseMohr-TraNEBjaergsyndrome(MTS)alsoknownasdystonia-deafnesssyndrome(DDS)orX-linkedprogressivedeafnestype1(DFN-1). Thisisarecessiveneurodegenerativesyndromecharacterisedbyprogressivedeafness,dystonia,spasticity,dysphagia,mentaldeterioration,paranoiaandcorticalblindness.DefectsinTIMM8AarealsothecauseofJensensyndrom,alsoknownasopticoacousticnerveatrophywithdementia.ThisisanX-linkeddiseasecharacterisedbydeafness,blindessandmuscleweakness.
BatchNo.Seeproductlabel
Unitsize100µg
AntigenPartialrecombinantproteinofhumanDeafnessdystoniaprotein1(aa9to98)withaGSTtag.
Clone2F11
OtherNamesMitochondrialimportinnermembranetranslocasesubunitTim8A;X-linkeddeafnessdystoniaprotein;TIMM8A;DDP;DDP1;TIM8A
AccessionTIM8A_HUMAN
ProducedinMouse
PurityProteinGpurifiedimmunoglobulin
ApplicationsThisantibodyisrecommendedforWB,IHCandsandwichELISA.ForIHC,aconcentrationof3µg/mlisrecommended.Biosensisrecommendsoptimaldilutions/concentrationsshouldbedeterminedbytheenduser.
SpecificitySpecificityhasbeenconfirmedbyWBanddirectELISAagainsttheantigen.
Cross-reactivityHuman.Otherspecieshavenotbeentested.
BlastitToseethesharedidentitybetweendifferentspeciesorotherproteins,followthelinkintheAccessionfield,selectthesequencethatyouareinterestedinandcopyandpasteitHEREandblast/formatit.
FormLyophilisedfromPBSpH7.2
ReconstitutionReconstitutein100µlofsterilewater.Centrifugetoremoveanyinsolublematerial.
StorageAfterreconstitutionkeepaliquotsat-20ºCforhigherstABIlityorat4ºCwithanappropriateantibacterialagent.Glycerol(1:1)maybeaddedforadditionalstability.Avoidrepetitivefreeze/thawcycles.
ExpiryDate12monthsafterpurchase
品牌介绍

Biosensis专注于神经科学领域的抗体和试剂的开发,特别是神经营养因子和神经营养因子受体。 近30年来,Biosensis一直是该领域的全球领航者和OEM供应商。除神经营养因子,我们的神经科学产品组合还被广泛用于神经退行性疾病、神经发育和神经代谢的研究。重点研究领域包括阿尔茨海默氏症(AD)、帕金森氏症(PD)和肌萎缩侧索硬化(ALS)疾病,以及自噬和代谢应激障碍,包括肥胖、代谢综合征的研究、神经免疫学和炎症。

自营商城图标
厂家直采
全球直采 正品优价
正品保障图标
正品保障
厂家直发 有线跟踪
解放采购图标
正规清关
CIF100%正规报关,提供发票
及时交付图标
及时交付
限时必达 不达必赔