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 |
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。