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Mouse Anti-TIMM8A Recombinant Antibody (CBYJT-3181) (CBMAB-T2455-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to TIMM8A (Translocase Of Inner Mitochondrial Membrane 8A). The antibody can be used for immunoassay techniques, such as ELISA, IHC, WB.
See all TIMM8A antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYJT-3181
Antibody Isotype
IgG2a, κ
Application
ELISA, IHC, WB

Basic Information

Immunogen
Partial recombinant corresponding to aa9-97 from human TIMM8A (NP_004076) with GST tag. MW of the GST tag alone is 26kD
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.2
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 9-97

Target

Full Name
translocase of inner mitochondrial membrane 8 homolog A (yeast)
Introduction
TIMM8A is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. TIMM8A 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 TIMM8A 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.
Entrez Gene ID
UniProt ID
Alternative Names
Translocase Of Inner Mitochondrial Membrane 8A; X-Linked Deafness Dystonia Protein; Deafness Dystonia Protein 1; DDP1; DDP; Translocase Of Inner Mitochondrial Membrane 8 (Yeast) Homolog A; Translocase Of Inner Mitochondrial Membrane 8 Homolog A (Yeast); Mitochondrial Import Inner Membrane Translocase Subunit Tim8 A
Function
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.
Biological Process
Biological Process nervous system developmentSource:ProtInc1 Publication
Biological Process protein insertion into mitochondrial inner membraneSource:BHF-UCL1 Publication
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mohr-Tranebjaerg syndrome (MTS):
An X-linked recessive disorder characterized by postlingual sensorineural deafness with onset in early childhood, dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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