Mouse anti-Human Frataxin Monoclonal Antibody | anti-FXN antibody
Mouse Anti Human Frataxin
1mg/ml containing PBS, pH-7.4, 10% Glycerol and 0.02% Sodium Azide.
For periods up to 1 month store at 4 degree C, for longer periods of time, store at -20 degree C. Prevent freeze thaw cycles.
NCBI and Uniprot Product Information
NCBI Description
This nuclear gene encodes a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA from 8-33 repeats to >90 repeats results in Friedreich ataxia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2016]
Uniprot Description
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: EC 1.16.3.1; Mitochondrial; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 9q21.11
Cellular Component: cytosol; mitochondrial matrix; mitochondrion
Molecular Function: 2 iron, 2 sulfur cluster binding; ferric iron binding; ferrous iron binding; ferroxidase activity; iron-sulfur cluster binding; protein binding
Biological Process: cellular iron ion homeostasis; iron incorporation into metallo-sulfur cluster; negative regulation of apoptosis; positive regulation of catalytic activity; positive regulation of cell growth; positive regulation of cell proliferation; positive regulation of lyase activity; protein autoprocessing; response to iron ion
Disease: Friedreich Ataxia 1
Research Articles on FXN
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Product Notes
The FXN fxn (Catalog #AAA146706) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Mouse Anti Human Frataxin reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Frataxin can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Flow Cytometry (FC/FACS), Immunocytochemistry (ICC), Immunofluorescence (IF). Researchers should empirically determine the suitability of the FXN fxn for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Frataxin, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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