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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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Products
Proteins (18)
Over-Expression Lysate (4)
Recombinant (14)
FXN / Frataxin (18)
No (16)
Yes (2)
10His, N-terminus (1)
10His, N-terminus + Myc, C-terminus (1)
10His-SUMO, N-terminus + Myc, C-terminus (1)
6His, N-terminus (4)
GST (1)
GST, N-terminus (1)
His (1)
His-SUMO (1)
His-T7 (1)
Myc-DDK (Flag) (5)
Cynomolgus monkey (1)
Human (10)
Mouse (4)
Rat (2)
293T Cells (2)
E. coli (10)
HEK 293 Cells (3)
Wheat Germ Extract (2)
Yeast (1)
Purified (7)
FXN / Frataxin Protein
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E. coli
6His, N-terminus
18.3 kD
100 µg/$598; 20 µg/$405; 1 mg/$1,679
FXN / Frataxin Protein - Purified recombinant protein FXN was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
18.8 kDa
20 µg/$1,107
FXN / Frataxin Protein
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E. coli
His
100 µg/$411; 500 µg/$985; 20 µg/$276
FXN / Frataxin Protein
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Yeast
6His, N-terminus
16.4 kD
1 mg/$1,683; 100 µg/$499; 20 µg/$364
FXN / Frataxin Protein - Recombinant Frataxin By SDS-PAGE
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E. coli
6His, N-terminus
22.4 kDa
10 µg/$276; 50 µg/$412; 100 µg/$657; 200 µg/$822; 1 mg/$1,905; 500 µg/$1,455; 5 mg/$2,871; 2 mg/$2,090
FXN / Frataxin Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
6His, N-terminus
19.9 kDa
1 mg/$1,679; 100 µg/$500; 20 µg/$364
FXN / Frataxin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
19.09 kDa
100 µg/$494
FXN / Frataxin Protein - 12.5% SDS-PAGE of human FXN stained with Coomassie Blue
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Wheat Germ Extract
GST
49.5 kDa
10 µg/$479; 25 µg/$670
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E. coli
His-SUMO
30.4 kD
1 mg/$1,679; 100 µg/$500; 20 µg/$364
FXN / Frataxin Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
FXN / Frataxin Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
10His, N-terminus
1 mg/$1,679; 100 µg/$500; 20 µg/$364
FXN / Frataxin Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
None
1 mg/$1,756; 100 µg/$611; 20 µg/$447
FXN / Frataxin Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
10His, N-terminus + Myc, C-terminus
1 mg/$1,355; 100 µg/$420; 20 µg/$323
FXN / Frataxin Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
10His-SUMO, N-terminus + Myc, C-terminus
1 mg/$1,355; 100 µg/$420; 20 µg/$323
FXN / Frataxin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
19.09 kDa
20 µg/$150
FXN / Frataxin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
23.14 kDa
20 µg/$150
FXN / Frataxin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
23.14 kDa
100 µg/$494
FXN / Frataxin Protein - Recombinant Frataxin By SDS-PAGE
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E. coli
His-T7
19.8 kDa
10 µg/$273; 50 µg/$392; 100 µg/$625; 200 µg/$782; 1 mg/$1,847; 500 µg/$1,404; 5 mg/$2,807; 2 mg/$2,029
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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

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).