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TIMM8A elisa kit :: Human Mitochondrial import inner membrane translocase subunit Tim8 A (TIMM8A) ELISA Kit

Scan QR to view Datasheet Catalog #    MBS7211722
Typical Standard Curve/Testing Data
Unit / Price
48-Strip-Wells  /  $470 +1 FREE 8GB USB
96-Strip-Wells  /  $675 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,925 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,490 +6 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Mitochondrial import inner membrane translocase subunit Tim8 A (TIMM8A), ELISA Kit

 Also Known As   

Human Mitochondrial import inner membrane translocase subunit Tim8 A (TIMM8A) ELISA Kit

 Product Gene Name   

TIMM8A elisa kit

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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  Sample Manual Insert    Download PDF Manual View PDF Manual
 Request for Current Manual Insert    Request Current Manual
 OMIM    311150
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 Species Reactivity    Human
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 Specificity    This assay has high sensitivity and excellent specificity for detection of TIMM8A. No significant cross-reactivity or interference between TIMM8A and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between TIMM8A and all the analogues, therefore, cross reaction may still exist in some cases.
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 Samples    Serum, plasma, cell culture supernatants, body fluid and tissue homogenate
 Assay Type    Quantitative Competitive
 Sensitivity    0.1 ng/mL.
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 Preparation and Storage    Store all reagents at 2-8 degree C.
 Sample Preparation    We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team
 Product Note    Our ELISA 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.
 Other Notes    Small volumes of TIMM8A elisa kit 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    MBS7211722 is a ready-to-use microwell, strip plate Quantitative Competitive ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Mitochondrial import inner membrane translocase subunit Tim8 A (TIMM8A) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing TIMM8A, with an estimated sensitivity of 0.1 ng/mL. The ELISA analytical biochemical technique of the MBS7211722 kit is based on TIMM8A antibody-TIMM8A antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect TIMM8A antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, TIMM8A. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions such as Serum, plasma, cell culture supernatants, body fluid and tissue homogenate. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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Product Description specifically for TIMM8A elisa kit

   Intended Uses: This TIMM8A ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human TIMM8A. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

Principle of the Assay: TIMM8A ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-TIMM8A antibody and an TIMM8A-HRP conjugate. The assay sample and buffer are incubated together with TIMM8A-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the TIMM8A concentration since TIMM8A from samples and TIMM8A-HRP conjugate compete for the anti-TIMM8A antibody binding site. Since the number of sites is limited, as more sites are occupied by TIMM8A from the sample, fewer sites are left to bind TIMM8A-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The TIMM8A concentration in each sample is interpolated from this standard curve.
 Product Categories/Family for TIMM8A elisa kit    Signal Transduction
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 Typical Standard Curve/Testing Data of TIMM8A elisa kit    TIMM8A elisa kit Typical Standard Curve/Testing Data image
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Sample Manual Insert of MBS7211722. Click to request current manual
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NCBI/Uniprot data below describe general gene information for TIMM8A. It may not necessarily be applicable to this product.
 NCBI GI #    225543159
 NCBI GeneID    1678
 NCBI Accession #    NP_001139423.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001145951.1 [Other Products]
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 UniProt Secondary Accession #    Q6IRW6; B2R5A6 [Other Products]
 UniProt Related Accession #    O60220 [Other Products]
 Molecular Weight    10,998 Da
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 NCBI Official Full Name    mitochondrial import inner membrane translocase subunit Tim8 A isoform 2
 NCBI Official Synonym Full Names    translocase of inner mitochondrial membrane 8 homolog A (yeast)
 NCBI Official Symbol    TIMM8A [Similar Products]
 NCBI Official Synonym Symbols   
DDP; MTS; DDP1; DFN1; TIM8
[Similar Products]
 NCBI Protein Information    mitochondrial import inner membrane translocase subunit Tim8 A; deafness/dystonia peptide; deafness dystonia protein 1; X-linked deafness dystonia protein
 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 [Similar Products]
 UniProt Synonym Gene Names    DDP; DDP1; TIM8A [Similar Products]
 UniProt Entry Name    TIM8A_HUMAN
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 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    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. Defects in TIMM8A are the cause of Mohr-Tranebjaerg syndrome (MTS); 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); also known as opticoacoustic nerve atrophy with dementia. This X-linked disease is characterized by deafness, blindness and muscle weakness. Belongs to the small Tim family.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: Xq22.1

Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial intermembrane space

Molecular Function: metal ion binding

Biological Process: nervous system development; cellular protein metabolic process; protein targeting to mitochondrion

Disease: Mohr-tranebjaerg Syndrome; Opticoacoustic Nerve Atrophy With Dementia
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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.
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 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.
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Products associated with TIMM8A elisa kitPathways associated with TIMM8A elisa kit
 Reference Product  PubMed Publications
 TIMM13 elisa kit  >9 publications with TIMM8A and TIMM13
 TIMM23 elisa kit  >3 publications with TIMM8A and TIMM23
 SLC25A12 elisa kit  >1 publications with TIMM8A and SLC25A12
 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 elisa kitOrgans/Tissues associated with TIMM8A elisa kit
 Disease Name  Pubmed Publications
 Mohr-Tranebjaerg syndrome Antibodies  >27 publications with TIMM8A and Mohr-Tranebjaerg syndrome
 Atrophy Antibodies  >11 publications with TIMM8A and Atrophy
 Neurodegenerative Diseases Antibodies  >8 publications with TIMM8A and Neurodegenerative Diseases
 Movement Disorders Antibodies  >6 publications with TIMM8A and Movement Disorders
 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
 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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