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anti-FXN antibody :: Rabbit anti-Mouse Frataxin Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS618238 anti-FXN antibody
Unit / Price
0.05 mg  /  $455 +1 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   

Frataxin (FXN), Polyclonal Antibody

 Also Known As   

Frataxin (FXN)

 Product Synonym Names    Anti -Frataxin (FXN)
 Product Gene Name   

anti-FXN antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    chromosome: 9; Location: 9q21.11
 3D Structure    ModBase 3D Structure for O96034
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Mouse
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 Specificity    Recognizes mouse Frataxin (FXN).
 Purity/Purification    Affinity Purified
Purified by immunoaffinity chromatography.
 Form/Format    Supplied as a liquid in PBS, pH 7.5, 0.1% BSA.
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 Immunogen    A synthetic peptide corresponding to 20aa within the C-terminus of mouse FXN (KLH).
 Species Sequence Homology    Human, 94%
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 Preparation and Storage    May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
 Other Notes    Small volumes of anti-FXN 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.
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Product Description specifically for anti-FXN antibody

   Elemental iron is required for a variety of normal cellular functions and vital for proper growth and development. However, natural iron is quite insoluble and excess iron is harmful, since it can catalyze the formation of potentially damaging reactive oxygen species. Humans also have very limited capacity to excrete iron. Therefore, cells have developed mechanisms to improve solubility of iron and to control intracellular iron levels at the point of absorption in the intestine and other tissue. Several proteins including Ferritin, transferrin (Tf), transferrin receptors (TfRs), and iron regulatory proteins (IRPs), iron transporter (NRMAP2/DMT1/DCT1) etc. play a key role in iron metabolism. Some genes involved in iron-metabolism are associated with genetic disorders such as Friedreich's Ataxia (Frataxin), genetic hemochromatosis (HFE), and Sex-linked anemia (Hephaestin). Friedrich ataxia (FA), a degenerative disease characterized by progressive ataxia and hypertrophic cardiomyopathy leading to premature death, is cause by a deficiency in frataxin (Fxn). Fxn (mouse 207 aa, human 210 aa major isoform A; alternatively transcribed minor forms A1, 196 aa, and 171-aa isoform B) is a nuclear-encoded and highly conserved mitochondrial membrane protein. Fxn is expressed in tissues with highly metabolic activity, such as heart, liver, and brown fat. In FA, Fxn levels are greatly reduced due to inhibition of its transcription by expansion of an intronic GAA repeat. Defects in yeast Fxn produce mitochondria iron-overload. It is suggested that Fxn could play a role in mitochondrial iron storage.
 Product Categories/Family for anti-FXN antibody    Antibodies; Abs to Proteins
 Applications Tested/Suitable for anti-FXN antibody   

ELISA (EL/EIA), Western Blot (WB)

 Application Notes for anti-FXN antibody    Suitable for use in ELISA and Western Blot.
Dilution: Western Blot: 1-10ug/ml. Using ECL.
ELISA: 1:100,000. Using 50-100ng F6100-15 Frataxin, Mouse, Control Peptide/well.
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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 #    4100063
 NCBI GeneID    2395
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 UniProt Primary Accession #    O96034 [Other Products]
 UniProt Related Accession #    O96034; Q16595 [Other Products]
 Molecular Weight    7,201 Da [Similar Products]
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 NCBI Official Full Name    frataxin
 NCBI Official Synonym Full Names    frataxin
 NCBI Official Symbol    FXN [Similar Products]
 NCBI Official Synonym Symbols   
FA; X25; CyaY; FARR; FRDA; MGC57199; FXN
[Similar Products]
 NCBI Protein Information    frataxin, mitochondrial; OTTHUMP00000215688; friedreich ataxia protein
 UniProt Protein Name    Frataxin
 Protein Family    Frataxin
 UniProt Gene Name    x25 [Similar Products]
 UniProt Entry Name    O96034_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]
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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; Motility/polarity/chemotaxis; EC 1.16.3.1

Chromosomal Location of Human Ortholog: 9q21.11

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function: ferroxidase activity; 2 iron, 2 sulfur cluster binding; 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; proprioception; positive regulation of transferase activity; 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; negative regulation of apoptosis; positive regulation of oxidoreductase activity; heme biosynthetic process; oxidative phosphorylation

Disease: Friedreich Ataxia 1
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 Research Articles on FXN    1. these results suggest an important role for eEF1A2 in driving cap-independent translation of utrophin A in skeletal muscle.
2. there is a a severe depletion of CTCF in the 5'UTR of the FXN gene in FRDA, and coincident heterochromatin formation involving the +1 nucleosome via enrichment of H3K9me3 and recruitment of heterochromatin protein 1
3. Data show that -terminus region is flexible and intrinsically unfolded in human frataxin.
4. frataxin deficiency triggers inflammatory changes and death of Schwann cells that is inhibitable by inflammatory and anti-apoptotic drugs.
5. Molecular genetic studies revealed that all Friedreich's ataxia patients in the Paphos district of Cyprus had the most common mutation, a homozygous expansion of the GAA triplet repeat in the first intron of the frataxin gene.
6. Azelaoyl PAF significantly increases the intracellular frataxin levels by twofold in the neuroblastoma cell line SKNBE and fibroblasts from FRDA patients and Azelaoyl PAF increases frataxin protein through a transcriptional mechanism.
7. The PPARG pathway is dysregulated in frataxin deficient mice and Friedreich's ataxia patients.
8. Regulatory effects of recombinant human erythropoietin occur at the post-translational level.
9. identification nof a novel point mutation (c.165 + 1 G ] A) which involves the consensus GT dinucleotide in the 3'splice acceptor site of intron 1 of the frataxin gene in two compound heterozygous siblings with Friedreich ataxia
10. We did not find any mutations or polymorphisms, nor expansion of the intronic trinucleotide repeat of the frataxin gene in refractory anemia with ring sideroblasts
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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 anti-FXN antibodyDiseases associated with anti-FXN antibody
 Products by Pathway  Pathway Diagram
 HIF-2-alpha Transcription Factor Network Pathway antibodies  HIF-2-alpha Transcription Factor Network Pathway Diagram
 Disease Name  Pubmed Publications
 Friedreich Ataxia Antibodies  >58 publications with FXN and Friedreich Ataxia
 Neurodegenerative Diseases Antibodies  >47 publications with FXN and Neurodegenerative Diseases
 Diabetes Mellitus Antibodies  >5 publications with FXN and Diabetes Mellitus
 Vascular Diseases Antibodies  >2 publications with FXN and Vascular Diseases
 Inflammation Antibodies  >2 publications with FXN and Inflammation
 Myocardial Infarction Antibodies  >2 publications with FXN and Myocardial Infarction
 Myocardial Ischemia Antibodies  >2 publications with FXN and Myocardial Ischemia
 Diabetes Mellitus, Type 1 Antibodies  >2 publications with FXN and Diabetes Mellitus, Type 1
 Cardiomyopathies Antibodies  >2 publications with FXN and Cardiomyopathies
 Pneumonia Antibodies  >1 publications with FXN and Pneumonia
Organs/Tissues associated with anti-FXN antibody
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >15 publications with FXN and Blood
 Brain Antibodies  >7 publications with FXN and Brain
 Eye Antibodies  >5 publications with FXN and Eye
 Muscle Antibodies  >4 publications with FXN and Muscle
 Skin Antibodies  >3 publications with FXN and Skin
 Vascular Antibodies  >3 publications with FXN and Vascular
 Heart Antibodies  >2 publications with FXN and Heart
 Lung Antibodies  >2 publications with FXN and Lung
 Placenta Antibodies  >1 publications with FXN and Placenta
 Intestine Antibodies  >1 publications with FXN and Intestine
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