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TIMM8A Antibody – Mouse Monoclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6134204

MBS6134204 is an antibody targeting TIMM8A. Also known as TIMM8A (Mitochondrial Import Inner Membrane Translocase Subunit Tim8 A, Deafness Dystonia Protein 1, X-linked Deafness Dystonia Protein, DDP, DDP1, TIM8A) (AP). Reactive in Human. Host: Mouse. More details

$995 / 0.1 mL +1 FREE 8GB USB
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Sample Manual Insert View PDF
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Testing Data View PDF
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Certificate of Analysis (COA) View PDF
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QC Report View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Protocol View PDF

Product Overview

Online Datasheet in PDF
Product Name
TIMM8A, Monoclonal Antibody
Full Product Name
TIMM8A (Mitochondrial Import Inner Membrane Translocase Subunit Tim8 A, Deafness Dystonia Protein 1, X-linked Deafness Dystonia Protein, DDP, DDP1, TIM8A) (AP)
Product Gene Name
anti-TIMM8A antibody
Clonality
Monoclonal
Isotype
IgG2a,k
Clone Number
2F11
Host
Mouse
Species Reactivity
Human
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).
Purity/Purification
Purified by Protein A Affinity Chromatography.
Specificity
Recognizes human TIMM8A.
Preparation and Storage
Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.
Molecular Weight
13.4kDa (120aa) confirmed by MALDI-TOF
OMIM
U66035 mRNA
3D Structure
ModBase 3D Structure for O60220
Immunogen
Partial recombinant corresponding to aa9-97 from human TIMM8A (NP_004076) with GST tag. MW of the GST tag alone is 26kD.
Immunogen Sequence
AAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
Conjugate
AP

Product Categories/Family for anti-TIMM8A antibody

Antibodies; Disease Markers
Applications Tested/Suitable for anti-TIMM8A antibody
ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB)
Application Notes for anti-TIMM8A antibody
Sandwich ELISA: The detection limit is ~0.3ng/ml as a capture antibody
Applications are based on unconjugated antibody.

Western Blot (WB) of anti-TIMM8A antibody

Western Blot detection against Immunogen (35.53kD)
Mouse Anti-TIMM8A Monoclonal Antibody – Cat# MBS6134204

Western Blot (WB) of anti-TIMM8A antibody

TIMM8A monoclonal antibody Western Blot analysis of TIMM8A expression in HeLa.
Mouse Anti-TIMM8A Monoclonal Antibody, Western Blot (WB) – Cat# MBS6134204

Western Blot (WB) of anti-TIMM8A antibody

Western Blot analysis of TIMM8A expression in transfected 293T cell line by TIMM8A monoclonal antibody. Lane 1: TIMM8A transfected lysate (11kD). Lane 2: Non-transfected lysate.
Mouse Anti-TIMM8A Monoclonal Antibody, Western Blot (WB) – Cat# MBS6134204

Immunohistochemistry (IHC) of anti-TIMM8A antibody

Immunoperoxidase of monoclonal antibody to TIMM8A on formalin-fixed paraffin-embedded human liver. [antibody concentration 3ug/ml]
Mouse Anti-TIMM8A Monoclonal Antibody, Immunohistochemistry (IHC) – Cat# MBS6134204

Testing Data of anti-TIMM8A antibody

Detection limit for recombinant GST tagged TIMM8A is ~0.3ng/ml as a capture antibody.
Mouse Anti-TIMM8A Monoclonal Antibody, Testing Data – Cat# MBS6134204

Other Notes

Small volumes of anti-TIMM8A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Additional Details

NCBI/Uniprot data below describe general gene information for TIMM8A. It may not necessarily be applicable to this product.

NCBI GI #
4758152
NCBI GeneID
1678
NCBI Accession #
NP_004076
UniProt Primary Accession #
O60220
UniProt Secondary Accession #
Q6IRW6; B2R5A6
UniProt Related Accession #
O60220
GenBank Nucleotide #
NM_004085
NCBI Official Full Name
mitochondrial import inner membrane translocase subunit Tim8 A isoform 1
NCBI Official Synonym Full Names
translocase of inner mitochondrial membrane 8A
NCBI Official Symbol
TIMM8A
NCBI Official Synonym Symbols
DDP; MTS; DDP1; DFN1; TIM8
UniProt Protein Name
Mitochondrial import inner membrane translocase subunit Tim8 A
UniProt Synonym Protein Names
Deafness dystonia protein 1; X-linked deafness dystonia protein
Protein Family
Mitochondrial import inner membrane translocase
UniProt Gene Name
TIMM8A
UniProt Synonym Gene Names
DDP; DDP1; TIM8A
NCBI Protein Information
mitochondrial import inner membrane translocase subunit Tim8 A

NCBI Summary for TIMM8A

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.[provided by RefSeq, Mar 2009]
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UniProt Comments for TIMM8A

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.
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Research Articles on TIMM8A

1. The results of this study demonistrated that the syndrome of deafness-dystonia is cause by mutation of Timm8a.

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with anti-TIMM8A antibody

 Reference Product  PubMed Publications
 TIMM13 antibody  >9 publications with TIMM8A and TIMM13
 TIMM23 antibody  >3 publications with TIMM8A and TIMM23
 SLC25A12 antibody  >1 publications with TIMM8A and SLC25A12

Pathways associated with anti-TIMM8A antibody

 Products by Pathway  Pathway Diagram
 Metabolism Of Proteins Pathway antibodies  Metabolism Of Proteins Pathway Diagram
 Mitochondrial Protein Import Pathway antibodies  Mitochondrial Protein Import Pathway Diagram

Diseases associated with anti-TIMM8A antibody

 Disease Name  Pubmed Publications
 Mohr-Tranebjaerg syndrome Antibodies  >27 publications with TIMM8A and Mohr-Tranebjaerg syndrome
 Mitochondrial Diseases Antibodies  >14 publications with TIMM8A and Mitochondrial Diseases
 Eye Diseases Antibodies  >14 publications with TIMM8A and Eye Diseases
 Neurodegenerative Diseases Antibodies  >8 publications with TIMM8A and Neurodegenerative Diseases
 Death Antibodies  >4 publications with TIMM8A and Death
 Nerve Degeneration Antibodies  >3 publications with TIMM8A and Nerve Degeneration
 Cognition Disorders Antibodies  >2 publications with TIMM8A and Cognition Disorders
 Liver Diseases Antibodies  >1 publications with TIMM8A and Liver Diseases
 Jensen syndrome Antibodies  >1 publications with TIMM8A and Jensen syndrome

Organs/Tissues associated with anti-TIMM8A antibody

 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >6 publications with TIMM8A and Brain
 Muscle Antibodies  >3 publications with TIMM8A and Muscle
 Eye Antibodies  >3 publications with TIMM8A and Eye
 Liver Antibodies  >2 publications with TIMM8A and Liver
 Bone Antibodies  >1 publications with TIMM8A and Bone
 Blood Antibodies  >1 publications with TIMM8A and Blood
 Skin Antibodies  >1 publications with TIMM8A and Skin
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