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Home >> Antibodies >> Frataxin (Marker of Friedreich Ataxia)

Frataxin (Marker of Friedreich Ataxia)

Mouse Monoclonal Antibody [Clone FXN/2124]

Catalog #

Size

20 ug
100 ug
100 ug

Price (USD)

199.00
459.00
459.00

Formulation

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

Catalog No

Size

20 ug
100 ug
100 ug

Formulation

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

Price (USD)

199.00
459.00
459.00
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Formalin-fixed, paraffin-embedded human Pancreas stained with Frataxin Mouse Monoclonal Antibody (FXN/2124).

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

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

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Western Blot Analysis of Human Panc-2 and HepG2 cell lysate using Frataxin Mouse Monoclonal Antibody (FXN/2124).

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Analysis of Protein Array containing more than 19,000 full-length human proteins using Frataxin Mouse Monoclonal Antibody (FXN/2124).

Product Details

Synonyms

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

Positive Control

HepG2, Panc-2, HL-60 or HEK293 cells. Pancreas and Liver.

Known Applications & Suggested Dilutions

Western Blot, IHC-P         More Details

Western Blot (1-2ug/ml)
Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 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)
Optimal dilution for a specific application should be determined.

Immunogen

Recombinant fragment of human Frataxin (FXN) protein (around aa 57-210) (exact sequence is proprietary)

Cellular Localization

Cytoplasmic (Mitochondria)

Species Reactivity

Human.

Host / Ig Isotype

Mouse / IgG2b, kappa

Mol. Weight of Antigen

14kDa

Specificity & Comments

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.

Related Products



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.

Key References

  1. 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.

Bioinformatics

  •  Entrez Gene ID: 2395
  •  Gene Symbol: FXN
  •  SwissProt: Q16595
  •  Chromosome Location: 9q21.11
  •  Unigene: 20685
NBT02190