Frataxin Antibody (G-12): sc-518079

Frataxin Antibody (G-12) is a mouse monoclonal IgG1 kappa light chain antibody that detects Frataxin protein of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-Frataxin antibody (G-12) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. Frataxin, encoded by the frataxin gene located on chromosome 9q21.11, plays a crucial role in mitochondrial function, particularly in iron metabolism. Frataxin assembles into a stable homopolymer with remarkable iron-binding capabilities, capable of binding up to 10 iron atoms per molecule when expressed in E. coli. This iron-storage function is vital for maintaining mitochondrial health, as iron is essential for various cellular processes, including energy production through oxidative phosphorylation. Frataxin acts as an activator of oxidative phosphorylation, enhancing mitochondrial membrane potential and increasing cellular ATP levels. Frataxin expression is particularly prominent in tissues with high metabolic demands, such as the heart, liver, and brown adipose tissue, underscoring its importance in energy metabolism and overall cellular function.

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Frataxin Antibody (G-12) References:

1. Frataxin activates mitochondrial energy conversion and oxidative phosphorylation.  |  Ristow, M., et al. 2000. Proc Natl Acad Sci U S A. 97: 12239-43. PMID: 11035806
2. Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia.  |  Cavadini, P., et al. 2002. Hum Mol Genet. 11: 217-27. PMID: 11823441
3. Frataxin and mitochondrial FeS cluster biogenesis.  |  Stemmler, TL., et al. 2010. J Biol Chem. 285: 26737-26743. PMID: 20522547
4. Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia.  |  Chiang, S., et al. 2016. Clin Sci (Lond). 130: 853-70. PMID: 27129098
5. Frataxin deficiency induces lipid accumulation and affects thermogenesis in brown adipose tissue.  |  Turchi, R., et al. 2020. Cell Death Dis. 11: 51. PMID: 31974344
6. Frataxin controls ketone body metabolism through regulation of OXCT1.  |  Dong, YN., et al. 2022. PNAS Nexus. 1: pgac142. PMID: 36016708
7. Frataxin deficiency alters gene expression in Friedreich ataxia derived IPsc-neurons and cardiomyocytes.  |  Angulo, MB., et al. 2023. Mol Genet Genomic Med. 11: e2093. PMID: 36369844
8. Iron-frataxin involved in the protective effect of quercetin against alcohol-induced liver mitochondrial dysfunction.  |  Liu, J., et al. 2023. J Nutr Biochem. 114: 109258. PMID: 36587874
9. Expression and processing of mature human frataxin after gene therapy in mice.  |  Rojsajjakul, T., et al. 2024. Sci Rep. 14: 8391. PMID: 38600238
10. The Friedreich ataxia critical region spans a 150-kb interval on chromosome 9q13.  |  Montermini, L., et al. 1995. Am J Hum Genet. 57: 1061-7. PMID: 7485155
11. Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin.  |  Koutnikova, H., et al. 1997. Nat Genet. 16: 345-51. PMID: 9241270
12. Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes.  |  Campuzano, V., et al. 1997. Hum Mol Genet. 6: 1771-80. PMID: 9302253

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-Frataxin Antibody (G-12) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-Frataxin Antibody (G-12) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

Frataxin Antibody (G-12) Specifications

Alternate Product Name: FXN

Product Clone Name: G-12

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG₁ kappa light chain

Amino Acids: 56-210

Epitope Target: Frataxin

Epitope Species: human

Product Reactivity/Detection: Frataxin

Species Reactivity/Detection: human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG₁ kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for Frataxin Antibody (G-12): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, PE: Phycoerythrin