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FXN elisa kit :: Human frataxin ELISA Kit

Scan QR to view Datasheet Catalog #    MBS935917
Typical Testing Data/Standard Curve (for reference only)
Unit / Price
24-Strip-Wells (LIMIT 1)  /  $250 +1 FREE 8GB USB
48-Strip-Wells  /  $495 +1 FREE 8GB USB
96-Strip-Wells  /  $710 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,705 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,145 +6 FREE 8GB USB
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 Product Name   

frataxin (FXN), ELISA Kit

★Popular Item★
 Also Known As   

Human Frataxin, mitochondrial, FXN ELISA Kit

 Product Synonym Names    Human Frataxin; mitochondrial (FXN) ELISA kit; CyaY; FA; FARR; FRDA; MGC57199; X25; ; frataxin
 Product Gene Name   

FXN 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
 MBS935917 Testing Data    Testing Data PDF
 OMIM    606829
 3D Structure    ModBase 3D Structure for Q16595
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 Species Reactivity    Human
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 Specificity    This assay has high sensitivity and excellent specificity for detection of human FXN. No significant cross-reactivity or interference between human FXN and analogues was observed.
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 Samples    Serum, plasma, tissue homogenates, Cell lysates
 Assay Type    Sandwich
 Detection Range    23.5 pg/ml -1500 pg/ml.
 Sensitivity    The minimum detectable dose of human FXN is typically less than 5.8 pg/ml. The sensitivity of this assay, or Lower Limit of Detection (LLD) was defined as the lowest protein concentration that could be differentiated from zero. It was determined the mean O.D value of 20 replicates of the zero standard added by their three standard deviations.
 Intra-assay Precision    Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess.
 Inter-assay Precision    Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.
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 Preparation and Storage    Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Product Note    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.
 Other Notes    Small volumes of FXN 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 FXN purchase    MBS935917 is a ready-to-use microwell, strip plate Sandwich ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the frataxin (FXN) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range of 23.5 pg/ml -1500 pg/ml in biological research samples containing FXN, with an estimated sensitivity of The minimum detectable dose of human FXN is typically less than 5.8 pg/ml. The sensitivity of this assay, or Lower Limit of Detection (LLD) was defined as the lowest protein concentration that could be differentiated from zero. It was determined the mean O.D value of 20 replicates of the zero standard added by their three standard deviations. The ELISA analytical biochemical technique of the MBS935917 kit is based on FXN antibody-FXN antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect FXN antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, FXN. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions such as Serum, plasma, tissue homogenates, Cell lysates. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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Related Product Information for FXN elisa kit

Principle of the assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for FXN has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any FXN present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for FXN is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of FXN bound in the initial step. The color development is stopped and the intensity of the color is measured.
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 Typical Testing Data/Standard Curve (for reference only) of FXN elisa kit    FXN elisa kit Typical Testing Data/Standard Curve (for reference only) image
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Sample Manual Insert of MBS935917. Click to request current manual
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NCBI/Uniprot data below describe general gene information for FXN. It may not necessarily be applicable to this product.
 NCBI GI #    31077081
 NCBI GeneID    2395
 NCBI Accession #    NP_000135.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000144.4 [Other Products]
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 UniProt Primary Accession #    Q16595 [Other Products]
 UniProt Secondary Accession #    O15545; O95656; Q15294; Q5VZ01; A8MXJ6; C9JJ89 [Other Products]
 UniProt Related Accession #    Q16595 [Other Products]
 Molecular Weight    23,135 Da
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 NCBI Official Full Name    frataxin, mitochondrial isoform 1 preproprotein
 NCBI Official Synonym Full Names    frataxin
 NCBI Official Symbol    FXN [Similar Products]
 NCBI Official Synonym Symbols   
[Similar Products]
 NCBI Protein Information    frataxin, mitochondrial; Friedreich ataxia protein
 UniProt Protein Name    Frataxin, mitochondrial
 UniProt Synonym Protein Names   
Friedreich ataxia protein; Fxn
 Protein Family    Frataxin
 UniProt Gene Name    FXN [Similar Products]
 UniProt Synonym Gene Names    FRDA; X25; Fxn; i-FXN [Similar Products]
 UniProt Entry Name    FRDA_HUMAN
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 NCBI Summary for FXN    This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2009]
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 UniProt Comments for FXN    FXN: Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1. Defects in FXN are the cause of Friedreich ataxia (FRDA). FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. Belongs to the frataxin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; EC; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 9q21.11

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function: 2 iron, 2 sulfur cluster binding; ferroxidase activity; protein binding; ferric iron binding; ferrous iron binding; iron-sulfur cluster binding

Biological Process: mitochondrion organization and biogenesis; negative regulation of multicellular organism growth; cellular iron ion homeostasis; positive regulation of metalloenzyme activity; positive regulation of transferase activity; proprioception; negative regulation of organ growth; positive regulation of cell growth; adult walking behavior; embryonic development ending in birth or egg hatching; protein autoprocessing; iron incorporation into metallo-sulfur cluster; positive regulation of lyase activity; positive regulation of cell proliferation; aerobic respiration; ion transport; response to iron ion; positive regulation of oxidoreductase activity; negative regulation of apoptosis; oxidative phosphorylation; heme biosynthetic process

Disease: Friedreich Ataxia 1
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 Research Articles on FXN    1. This early failure, aggravating frataxin deficiency in a specifically vulnerable human cell population, indicates a developmental component in Friedreich ataxia .
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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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Pathways associated with FXN elisa kitDiseases associated with FXN elisa kit
 Products by Pathway  Pathway Diagram
 HIF-2-alpha Transcription Factor Network Pathway antibodies  HIF-2-alpha Transcription Factor Network Pathway Diagram
 Metabolism Of Proteins Pathway antibodies  Metabolism Of Proteins Pathway Diagram
 Mitochondrial Protein Import Pathway antibodies  Mitochondrial Protein Import Pathway Diagram
 Disease Name  Pubmed Publications
 Friedreich Ataxia Antibodies  >72 publications with FXN and Friedreich Ataxia
 Heart Diseases Antibodies  >7 publications with FXN and Heart Diseases
 Death Antibodies  >4 publications with FXN and Death
 Hypertrophy Antibodies  >4 publications with FXN and Hypertrophy
 Body Weight Changes Antibodies  >2 publications with FXN and Body Weight Changes
 Atrophy Antibodies  >2 publications with FXN and Atrophy
 Neoplasms Antibodies  >2 publications with FXN and Neoplasms
 Myocardial Infarction Antibodies  >2 publications with FXN and Myocardial Infarction
 Bradycardia Antibodies  >2 publications with FXN and Bradycardia
 Cardiomyopathies Antibodies  >2 publications with FXN and Cardiomyopathies
Organs/Tissues associated with FXN elisa kit
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >17 publications with FXN and Blood
 Heart Antibodies  >10 publications with FXN and Heart
 Muscle Antibodies  >10 publications with FXN and Muscle
 Brain Antibodies  >8 publications with FXN and Brain
 Liver Antibodies  >4 publications with FXN and Liver
 Skin Antibodies  >2 publications with FXN and Skin
 Embryonic Tissue Antibodies  >2 publications with FXN and Embryonic Tissue
 Vascular Antibodies  >1 publications with FXN and Vascular
 Eye Antibodies  >1 publications with FXN and Eye
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