Overview

  • Product nameAnti-TIMM8A antibody
    See all TIMM8A primary antibodies
  • Description
    Mouse polyclonal to TIMM8A
  • Tested applicationsSuitable for: WB, ICC/IFmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Full length human TIMM8A protein (NP_004076.1).

  • Positive control
    • WB: HepG2 cell lysate and TIMM8A transfected 293T cell lysate. ICC/IF: Hela cell

Properties

Associated products

Applications

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.

Application Abreviews Notes
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 11 kDa.
ICC/IF Use a concentration of 10 µg/ml.

Target

  • FunctionMitochondrial 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.
  • Tissue specificityHighly 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.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the small Tim family.
  • DomainThe 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.
  • Cellular localizationMitochondrion inner membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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 images

  • 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.

References for Anti-TIMM8A antibody (ab88311)

ab88311 has not yet been referenced specifically in any publications.

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