概述

  • 产品名称Anti-TIMM8A抗体
    参阅全部 TIMM8A 一抗
  • 描述
    小鼠多克隆抗体to TIMM8A
  • 经测试应用适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length human TIMM8A protein (NP_004076.1).

  • 阳性对照
    • WB: HepG2 cell lysate and TIMM8A transfected 293T cell lysate. ICC/IF: Hela cell

性能

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应用

Our Abpromise guarantee covers the use of ab88311 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 11 kDa.
ICC/IF Use a concentration of 10 µg/ml.

靶标

  • 功能Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development.
  • 组织特异性Highly expressed in fetal and adult brain, followed by fetal lung, liver and kidney. Also expressed in heart, placenta, lung, liver, kidney, pancreas, skeletal muscle and heart.
  • 疾病相关Defects in TIMM8A are the cause of Mohr-Tranebjaerg syndrome (MTS) [MIM:304700]; also known as dystonia-deafness syndrome (DDS) or X-linked progressive deafness type 1 (DFN-1). It is a recessive neurodegenerative syndrome characterized by postlingual progressive sensorineural deafness as the first presenting symptom in early childhood, followed by progressive dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness.
    Defects in TIMM8A are the cause of Jensen syndrome (JENSS) [MIM:311150]; also known as opticoacoustic nerve atrophy with dementia. This X-linked disease is characterized by deafness, blindness and muscle weakness.
  • 序列相似性Belongs to the small Tim family.
  • 结构域The twin CX3C motif contains 4 conserved Cys residues that form 2 disulfide bonds in the mitochondrial intermembrane space. However, during the transit of TIMM8A from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across mitochondrial outer membrane.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • DDP 1 antibody
    • DDP antibody
    • DDP1 antibody
    • Deafness dystonia protein 1 antibody
    • Deafness/dystonia peptide antibody
    • DFN 1 antibody
    • DFN1 antibody
    • MGC12262 antibody
    • Mitochondrial import inner membrane translocase subunit Tim8 A antibody
    • MTS antibody
    • TIM 8A antibody
    • TIM8 antibody
    • TIM8A antibody
    • TIM8A_HUMAN antibody
    • TIMM 8A antibody
    • timm8a antibody
    • Translocase of inner mitochondrial membrane 8 homolog A antibody
    • X linked deafness dystonia protein antibody
    • X-linked deafness dystonia protein antibody
    see all

Anti-TIMM8A antibody 图像

  • Anti-TIMM8A antibody (ab88311) at 1 µg/ml + HepG2 cell lysate at 50 µg

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/5000 dilution

    Predicted band size : 11 kDa
    Observed band size : 13 kDa (why is the actual band size different from the predicted?)
  • All lanes : Anti-TIMM8A antibody (ab88311) at 1 µg/ml

    Lane 1 : TIMM8A transfected 293T cell lysate
    Lane 2 : non transfected 293T cell lysate

    Lysates/proteins at 50 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/5000 dilution

    Predicted band size : 11 kDa
    Observed band size : 11 kDa
  • ab88311, at 10 µg/ml, staining TIMM8A in Hela cell by Immunofluorescence.

Anti-TIMM8A antibody (ab88311)参考文献

ab88311 has not yet been referenced specifically in any publications.

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