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Frataxin (Marker of Friedreich Ataxia) Antibody [FXN/2124]

In Stock
Catalog Number Formulation Size Price
2395-MSM4-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$229.00
2395-MSM4-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$519.00
2395-MSM4-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$519.00
Flat Rate Domestic: $60 | Orders outside the US - Contact Us for Order Information | Ships next business day

Applications & Dilutions

Applications Tested Dillution Protocol Note
Immunohistochemistry (IHC)
1-2ug/ml
30 min at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes
Western Blot (WB)
2-4ug/ml

Summary

Frataxin is a nuclear-encoded mitochondrial protein implicated in Friedreich's ataxia (FRDA), a human autosomal recessive neurodegenerative disease. Lack of Frataxin causes iron to accumulate in the mitochondrial matrix suggesting that Frataxin is involved in mitochondrial iron homeostasis and possibly in iron transport. Frataxin has an alpha-beta fold consisting of two helices flanking an antiparallel beta sheet.

Product Properties & Targets

Antibody Type
Host
Mouse
Species Reactivity
Isotype / Light Chain
IgG2b / Kappa
Cellular Localization
Cytoplasm, Cytosol, Mitochondrion
Gene Name
FXN
Positive Control
HePG2, HL-60 or HEK293 cells. Pancreas and Liver., Panc-2
Immunogen
Recombinant fragment of human Frataxin (FXN) protein (around aa 57-210) (exact sequence is proprietary)
Alternate Names
Frataxin, mitochondrial, Friedreich ataxia protein, CyaY; d-FXN; FARR; Frataxin mature form; Frataxin (81-210); FRDA; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; X25

Database Links

Entrez Gene ID
SwissProt

Additional Information

Clone
FXN/2124
Chromosome Location
9q21.11
Mol. Weight of Antigen
14kDa

Functions

  • 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.

Key References

  • Suebpeng Y, Jetsrisuparb A, Fucharoen S, Tripatara A. Frataxin expression in reticulocytes of non-splenectomized and splenectomized patients with HbE--thalassaemia. Clin Biochem. 2016 Apr;49(6):463-466.

Storage & Stability

Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Limitations

This antibody is available for research use only and is not approved for use in diagnosis.

Supplied as

200ug/ml of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Warranty

There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.

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