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TIMM8A Polyclonal Antibody

规格: / 20μL / 60μL / 120μL / 200μL
价格: / ¥420 / ¥1010 / ¥1640 / ¥2365

货号:E-AB-18755

宿主: Rabbit

反应性: H,M,R

应用: IHC

  • 详情
  • Overview

    Synonyms DDP 1,DDP,DDP1,Deafness dystonia protein 1,Deafness/dystonia peptide,DFN 1,DFN1,MGC12262,Mitochondrial import inner membrane translocase subunit Tim8 A,MTS,TIM 8A,TIM8,TIM8A,TIM8A,TIMM 8A,timm8a,Translocase of inner mitochondrial membrane 8 homolog A,X linked deafness dystonia protein,X-linked deafness dystonia protein,TIMM8A抗体
    Swissprot O60220
    Source Rabbit
    Reactivity Human,Mouse,Rat
    Immunogen Fusion protein of human TIMM8A
    Application IHC(Detection kit: E-IR-R213)
    Recommended dilution IHC,,1:40-1:200
    Concentration 0.84mg/mL
    Clonality Polyclonal

    Properties

    Cellular localization Mitochondrion inner membrane.
    Isotype IgG
    Purification Affinity purification
    Conjugation Unconjugated
    Storage instructions Store at -20℃. Avoid freeze / thaw cycles.
    Storage buffer PBS with 0.05% NaN3 and 40% Glycerol,pH7.4
    Background This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
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