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FXN elisa kit :: Human frataxin ELISA Kit

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Catalog # MBS935917
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Typical Testing Data/Standard Curve (for reference only)
Product Name

frataxin (FXN), ELISA Kit

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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
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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MBS935917 Testing Data
Sequence Length
210
OMIM
606829
3D Structure
ModBase 3D Structure for Q16595
Species Reactivity
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.
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.
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 forFXNpurchase
MBS935917 is a ready-to-use microwell, strip plate 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 in biological research samples containing FXN. 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. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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.

Typical Testing Data/Standard Curve (for reference only) of FXN elisa kit
FXN elisa kit Typical Testing Data/Standard Curve (for reference only) image
Sample Manual Insert of MBS935917. Click to request current manual
NCBI/Uniprot data below describe general gene information for FXN. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
23,135 Da
NCBI Official Full Name
frataxin, mitochondrial isoform 1 preproprotein
NCBI Official Synonym Full Names
frataxin
NCBI Official Symbol
NCBI Official Synonym Symbols
FA; X25; CyaY; FARR; FRDA
  [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
UniProt Gene Name
UniProt Synonym Gene Names
FRDA; X25; Fxn; i-FXN  [Similar Products]
UniProt Entry Name
FRDA_HUMAN
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]
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 1.16.3.1; 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
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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