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蚂蚁淘在线 / 品牌中心 / Biosensis / Biosensis/Mouse monoclonal to human Deafness dystonia protein 1 [2F11]: IgG/M-831-100/100 µg
商品描述
| 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 |
| Unitsize | 100µg |
| Antigen | PartialrecombinantproteinofhumanDeafnessdystoniaprotein1(aa9to98)withaGSTtag. |
| Clone | 2F11 |
| OtherNames | MitochondrialimportinnermembranetranslocasesubunitTim8A;X-linkeddeafnessdystoniaprotein;TIMM8A;DDP;DDP1;TIM8A |
| Accession | TIM8A_HUMAN |
| Producedin | Mouse |
| Purity | ProteinGpurifiedimmunoglobulin |
| Applications | ThisantibodyisrecommendedforWB,IHCandsandwichELISA.ForIHC,aconcentrationof3µg/mlisrecommended.Biosensisrecommendsoptimaldilutions/concentrationsshouldbedeterminedbytheenduser. |
| Specificity | SpecificityhasbeenconfirmedbyWBanddirectELISAagainsttheantigen. |
| Cross-reactivity | Human.Otherspecieshavenotbeentested. |
| Blastit | Toseethesharedidentitybetweendifferentspeciesorotherproteins,followthelinkintheAccessionfield,selectthesequencethatyouareinterestedinandcopyandpasteitHEREandblast/formatit. |
| Form | LyophilisedfromPBSpH7.2 |
| Reconstitution | Reconstitutein100µlofsterilewater.Centrifugetoremoveanyinsolublematerial. |
| Storage | Afterreconstitutionkeepaliquotsat-20ºCforhigherstABIlityorat4ºCwithanappropriateantibacterialagent.Glycerol(1:1)maybeaddedforadditionalstability.Avoidrepetitivefreeze/thawcycles. |
| ExpiryDate | 12monthsafterpurchase |
