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FXN / Frataxin

frataxin

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.

Gene Name: frataxin
Synonyms: FXN, CyaY, Frataxin, mitochondrial, Friedreich ataxia protein, FARR, Friedreich ataxia, FA, FRDA, X25, Frataxin
Target Sequences: NM_000144 NP_000135.2 Q16595

Publications (3)

1
Characterization of a new N-terminally acetylated extra-mitochondrial isoform of frataxin in human erythrocytes. Guo L, Wang Q, Weng L, Hauser LA, Strawser CJ, Mesaros C, Lynch DR, Blair IA. Scientific reports. 2018 November;8:17043. [Full Text Article] [PubMed:30451920] [PMC:PMC6242848] Related Antibodies: LS-C197243.
2
Liquid Chromatography-High Resolution Mass Spectrometry Analysis of Platelet Frataxin as a Protein Biomarker for the Rare Disease Friedreich's Ataxia. Lili Guo, Qingqing Wang, Liwei Weng, Lauren A Hauser, Cassandra J Strawser, Agostinho G Rocha, Andrew Dancis, Clementina Mesaros, David R Lynch, Ian A Blair. Analytical Chemistry. 2018 February;90:2216-2223. [Full Text Article] [PubMed:29272104] [PMC:PMC5817373] Related Antibodies: LS-C197243.
3
Evaluation of antibodies for western blot analysis of frataxin protein isoforms. Liwei Weng, Qingqing Wang, Sixiang Yu, Xiaolu Yang, David R Lynch, Clementina Mesaros, Ian A Blair. Journal of immunological methods. 2019 Nov;474:112629. [Full Text Article] [PubMed:31279523] [PMC:PMC6829029] Related Antibodies: LS-C197243.

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ELISA Kits (6)
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FXN / Frataxin (6)
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FXN / Frataxin ELISA Kit
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Sandwich
96-Well Microplate
Human
23.44 - 1500 pg/ml
Colorimetric - 450nm (TMB)
Plasma, Serum
1 Plate/$771
FXN / Frataxin ELISA Kit
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Sandwich
96-Well Strip Plate
Rat
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum
1 Plate/$669
FXN / Frataxin ELISA Kit
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Sandwich
96-Well Strip Plate
Mouse
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Cell Lysates, Tissue Homogenates
1 Plate/$748
FXN / Frataxin ELISA Kit
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Sandwich
96-Well Strip Plate
Human
0.156 - 10 ng/nl
1 Plate/$669
FXN / Frataxin ELISA Kit
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Custom
96-Well Strip Plate
Bovine
1 Plate/$669
FXN / Frataxin ELISA Kit
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Custom
96-Well Strip Plate
Mouse
1 Plate/$669
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The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).