MAB1594
Anti-Frataxin Antibody, exon 4, clone 1G2
ascites fluid, clone 1G2, Chemicon®
Synonym(s):
Friedreich ataxia, Friedreich ataxia protein
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About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
biological source
mouse
Quality Level
antibody form
ascites fluid
antibody product type
primary antibodies
clone
1G2, monoclonal
species reactivity
human, rat, mouse
manufacturer/tradename
Chemicon®
technique(s)
ELISA: suitable
immunocytochemistry: suitable
immunofluorescence: suitable
western blot: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
dry ice
target post-translational modification
unmodified
Gene Information
human ... FXN(2395)
mouse ... Fxn(14297)
rat ... Fxn(499335)
General description
Frataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle (Campuzano, 1996; Koutnikova, 1997). Frataxin is expressed as a 30 kDa precursor (transient; 210 amino acids) that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase (MPP) to yield the mature protein (Koutnikova, 1998). The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20 kDa (aa 42-210). Further cleavage of the N-terminus of this 20 kDa intermediate by MPP results in the mature 18 kDa frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich′s ataxia, an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy. In the majority of cases of Friedreich′s ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription (Campuzano, 1997; Bidichandani, 1998). This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress (Foury, 1997; for review see Puccio, 2000).
Specificity
Expected to cross-react with human.
Human Frataxin. MAB1594 recognizes only isoforms of frataxin containing exon 4. On Western blots of normal human muscle, heart, cerebellum, and spinal cord extracts, MAB1594 recognizes a band migrating at approximately 18 kDa corresponding to processed frataxin (Campuzano, 1997). Slight cross reactivity with myosin may be observed by Western blot. Immunofluorescent labeling of HeLa cells with MAB1594 demonstrates that frataxin is predominantly localized in the mitochondria (Campuzano, 1997).
Immunogen
Epitope: exon 4
Full length human Frataxin fused to TrpE.
Application
Detect Frataxin using this Anti-Frataxin Antibody, exon 4, clone 1G2 validated for use in ELISA, IC, IF & WB.
Immunofluorescence:
1:100-1:1,000. Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to the subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody.
ELISA:
A previous lot of this antibody was used on ELISA.
Western blot (natural and recombinant protein):
1:5,000; mitochondrial preparations are recommended for consist signals (see Santos, 2001).
Optimal working dilutions must be determined by the end user.
1:100-1:1,000. Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to the subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody.
ELISA:
A previous lot of this antibody was used on ELISA.
Western blot (natural and recombinant protein):
1:5,000; mitochondrial preparations are recommended for consist signals (see Santos, 2001).
Optimal working dilutions must be determined by the end user.
Quality
Routinely evaluated by Western Blot on PC12 lysates.
Western Blot Analysis:
1:1000 dilution of this lot detected Frataxin on 10 μg of PC12 lysates.
Western Blot Analysis:
1:1000 dilution of this lot detected Frataxin on 10 μg of PC12 lysates.
Target description
~ 18 kDa
Physical form
Mouse monoclonal ascites IgG1κ in buffer containing liquid with no preservatives.
Storage and Stability
Stable for 1 years at -20°C in undiluted aliquots from date of receipt.
Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance
Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance
Analysis Note
Control
Liver, heart or skeletal muscle.
Liver, heart or skeletal muscle.
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Legal Information
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
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Storage Class Code
11 - Combustible Solids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Liwei Weng et al.
Journal of immunological methods, 474, 112629-112629 (2019-07-08)
Frataxin is the protein that is down-regulated in Friedreich ataxia (FRDA), an autosomal recessive genetic disease caused by an intronic GAA repeat expansion in intron-1 of the FXN gene. The GAA repeats result in epigenetic silencing of the FXN gene
Brigitte Sturm et al.
European journal of clinical investigation, 40(6), 561-565 (2010-05-12)
Friedreich's ataxia (FRDA) is a neurodegenerative disorder caused by decreased expression of the mitochondrial protein frataxin. Recently we showed in a clinical pilot study in Friedreich's ataxia patients that recombinant human erythropoietin (rhuEPO) significantly increases frataxin-expression. In this in vitro
H Khonsari et al.
Gene therapy, 23(12), 846-856 (2016-10-21)
Friedreich ataxia (FRDA) is a progressive neurodegenerative disease caused by deficiency of frataxin protein, with the primary sites of pathology being the large sensory neurons of the dorsal root ganglia and the cerebellum. FRDA is also often accompanied by severe
Daniel Fil et al.
Disease models & mechanisms, 13(7) (2020-06-27)
Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin (FXN). Most FRDA patients are homozygous for large expansions of GAA repeat sequences in intron 1 of FXN, whereas a fraction of patients
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