CyaY; d-FXN; FARR; Frataxin mature form; Frataxin (81-210); FRDA; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; X25

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2b, kappa

FXN/2124

Mouse

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.

Purified Ab with BSA and Azide at 200ug/ml OR Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

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.

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

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.

Immunohistology (Formalin-Fixed)

Immunohistology (Formalin-Fixed): 1-2ug/ml for 30 min at RT; Staining of formalin-fixed tissues requires boiling tissue sections in 10mM Citrate Buffer, pH 6.0, for 10-20 min followed by cooling at RT for 20 minutes.

Formalin-fixed, paraffin-embedded human Pancreas stained with Frataxin Mouse Monoclonal Antibody (FXN/2124).

Formalin-fixed, paraffin-embedded human Pancreas stained with Frataxin Mouse Monoclonal Antibody (FXN/2124).

SDS-PAGE Analysis Purified Frataxin Mouse Monoclonal Antibody (FXN/2124). Confirmation of Purity and Integrity of Antibody.

Analysis of Protein Array containing more than 19,000 full-length human proteins using Frataxin Mouse Monoclonal Antibody (FXN/2124). Z- and S- Score: The Z-score represents the strength of a signal that a monoclonal antibody (MAb) (in combination with a fluorescently-tagged anti-IgG secondary antibody) produces when binding to a particular protein on the HuProtTM array. Z-scores are described in units of standard deviations (SD's) above the mean value of all signals generated on that array. If targets on HuProtTM are arranged in descending order of the Z-score, the S-score is the difference (also in units of SD's) between the Z-score. S-score therefore represents the relative target specificity of a MAb to its intended target. A MAb is considered to specific to its intended target, if the MAb has an S-score of at least 2.5. For example, if a MAb binds to protein X with a Z-score of 43 and to protein Y with a Z-score of 14, then the S-score for the binding of that MAb to protein X is equal to 29.

14kDa

frataxin

frataxin, mitochondrial

Frataxin, mitochondrial

FRDA; X25; Fxn; i-FXN??[Similar Products]

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]

Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ 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 Fe2+ to Fe3+; 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.

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

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