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Mouse Anti-TIMM8A (C-terminus) Recombinant Antibody (CBFYM-3157) (CBMAB-M3354-FY)

This product is mouse antibody that recognizes TIMM8A. The antibody CBFYM-3157 can be used for immunoassay techniques such as: WB, IP.
See all TIMM8A antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
CBFYM-3157
Antibody Isotype
IgG1
Application
WB, IP

Basic Information

Immunogen
A KLH coupled peptide with the sequence: A-RRTPDYFL-Ome
Specificity
Mouse, Rat, Human
Antibody Isotype
IgG1
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!]

Format
Liquid
Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
C-terminus

Target

Full Name
translocase of inner mitochondrial membrane 8 homolog A (yeast)
Introduction
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 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.
Entrez Gene ID
Human1678
Mouse30058
Rat84383
UniProt ID
HumanO60220
MouseQ9WVA2
RatQ9WVA1
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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