TIMM8A Recombinant Protein

Name: TIMM8A Recombinant Protein; Research Grade; MyBioSource
Brand: MyBioSource
SKU: MBS204942
Price: 230 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS204942

TIMM8A 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… More details

$230 / 0.02 mg +1 FREE 8GB USB

Product Documentation

Sample Manual Insert View PDF

Testing Data View PDF

Certificate of Analysis (COA) View PDF

QC Report View PDF

Material Safety Data Sheet View PDF

Technical Datasheet View PDF

Protocol View PDF

Product Overview

Online Datasheet in PDF

Product Name

TIMM8A, Recombinant Protein

Full Product Name

TIMM8A, 1-97aa, Human, His tag, E Coli

Product Gene Name

TIMM8A recombinant protein

Product Synonym Names

Mitochondrial import inner membrane translocase subunit Tim8 A; Mitochondrial import inner membrane translocase subunit Tim8 A; DDP; DDP1; DFN1; MTS; TIM8

Sequence

MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD

Host

E Coli

Form/Format

Liquid. In 20mM Tris-HCl Buffer (pH 8.0) containing 0.15M NaCl, 30% glycerol, 1mM DTT

Purity/Purification

> 90% by SDS-PAGE

Concentration

0.25 mg/ml (determined by Bradford assay) (lot specific)

Preparation and Storage

Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.

Molecular Weight

13.4kDa (120aa) confirmed by MALDI-TOF

OMIM

U66035 mRNA

3D Structure

ModBase 3D Structure for O60220

Antigen Species

Human

Tag Information

His-tag

Product Categories/Family for TIMM8A recombinant protein

Signal Transduction

Applications Tested/Suitable for TIMM8A recombinant protein

SDS-PAGE

Product References and Citations for TIMM8A recombinant protein

Rothbauer u., et al. (2001) J. Biol. Chem. 276:37327-37334; Roesch K., et al. (2004) Hum. Mol. Genet. 13:2101-2111;

Other Notes

Small volumes of TIMM8A recombinant protein 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.

Searchable Terms for TIMM8A purchase

MBS204942 is a recombinant protein targeting TIMM8A. Also known as TIMM8A, 1-97aa, Human, His tag, E Coli. Host: E Coli.

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_004085.3

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]

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.

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 TIMM8A recombinant protein

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

Pathways associated with TIMM8A recombinant protein

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 TIMM8A recombinant protein

Disease Name	Pubmed Publications
Mohr-Tranebjaerg syndrome Antibodies	>29 publications with TIMM8A and Mohr-Tranebjaerg syndrome
Neurodegenerative Diseases Antibodies	>8 publications with TIMM8A and Neurodegenerative Diseases
Nerve Degeneration Antibodies	>3 publications with TIMM8A and Nerve Degeneration
Cognition Disorders Antibodies	>2 publications with TIMM8A and Cognition Disorders
Chromosome Breakage Antibodies	>1 publications with TIMM8A and Chromosome Breakage
Liver Diseases Antibodies	>1 publications with TIMM8A and Liver Diseases
Jensen syndrome Antibodies	>1 publications with TIMM8A and Jensen syndrome

Organs/Tissues associated with TIMM8A recombinant protein

Organ/Tissue Name	Pubmed Publications
Brain Antibodies	>7 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