Anti-Frataxin antibody [18A5DB1] (ab110328)
![Western blot - Anti-Frataxin antibody [18A5DB1] (ab110328)](https://www.abcam.com/ps/products/110/ab110328/Images/ab110328-123637-anti-frataxin-antibody-18a5db1-western-blot.jpg "Western blot - Anti-Frataxin antibody [18A5DB1] (ab110328)")
Key features and details
- Mouse monoclonal [18A5DB1] to Frataxin
- Suitable for: WB, ICC/IF, Flow Cyt, IHC-P
- Reacts with: Human
- Isotype: IgG1
Related conjugates and formulations
Overview
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Product name
Anti-Frataxin antibody [18A5DB1]
See all Frataxin primary antibodies -
Description
Mouse monoclonal [18A5DB1] to Frataxin -
Host species
Mouse -
Tested applications
Suitable for: WB, ICC/IF, Flow Cyt, IHC-Pmore details -
Species reactivity
Reacts with: Human -
Immunogen
Recombinant full length protein. This information is considered to be commercially sensitive.
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Positive control
- Recombinant Human Frataxin; normal Human fibroblasts (MRC5); HL60 cells. This antibody gave a positive result in IHC in the following FFPE tissue: Human colon adenocarcinoma.
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General notes
Although this antibody does work in Western blot, we have found that ab113691 typically gives a more robust signal. Therefore, if you plan on using this antibody in WB only, we recommend ab113691 as an alternative.
This antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact orders@abcam.com.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Product was previously marketed under the MitoSciences sub-brand.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C. Do Not Freeze. -
Storage buffer
pH: 7.5
Preservative: 0.02% Sodium azide
Constituent: HEPES buffered saline -
Concentration information loading... -
Purity
IgG fraction -
Purification notes
ab110328 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation. -
Clonality
Monoclonal -
Clone number
18A5DB1 -
Isotype
IgG1 -
Light chain type
kappa -
Research areas
Associated products
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Alternative Versions
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Compatible Secondaries
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Conjugation kits
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Isotype control
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Related Products
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab110328 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| WB | (4) |
Use a concentration of 3 - 5 µg/ml. Predicted molecular weight: 23 kDa. (A chemiluminescent detection system is highly recommended)
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| ICC/IF | (1) |
Use a concentration of 0.5 µg/ml.
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| Flow Cyt |
Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody. |
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| IHC-P |
Use a concentration of 10 µg/ml.
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| Notes |
|---|
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WB
Use a concentration of 3 - 5 µg/ml. Predicted molecular weight: 23 kDa. (A chemiluminescent detection system is highly recommended) |
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ICC/IF
Use a concentration of 0.5 µg/ml. |
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Flow Cyt
Use a concentration of 1 µg/ml. ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody. |
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IHC-P
Use a concentration of 10 µg/ml. |
Target
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Function
Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) 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 Fe(2+) to Fe(3+); 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. -
Tissue specificity
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. -
Involvement in disease
Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. -
Sequence similarities
Belongs to the frataxin family. -
Post-translational
modificationsProcessed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure. -
Cellular localization
Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. - Information by UniProt
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Database links
- Entrez Gene: 2395 Human
- Omim: 606829 Human
- SwissProt: Q16595 Human
- Unigene: 20685 Human
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Alternative names
- CyaY antibody
- d-FXN antibody
- FA antibody
see all
Images
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Western blot - Anti-Frataxin antibody [18A5DB1] (ab110328)Anti-Frataxin antibody [18A5DB1] (ab110328) at 5 µg/ml + Recombinant Human Frataxin at 0.0005 µg
Predicted band size: 23 kDa -
![Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [18A5DB1] (ab110328)](https://www.abcam.com/ps/products/110/ab110328/Images/ab110328-319836-anti-frataxin-antibody-18a5db1-immunofluorescence.jpg)
Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [18A5DB1] (ab110328)Image courtesy of Dr George AllenImmunofluorescent analysis of paraformaldehyde-fixed, 0.2% NP-40 permeabilized SH-SY5Y (human neuroblastoma cell line from bone marrow) cells labeling Frataxin with ab110328 at 1/500 dilution, followed by Goat anti-MouseAlexaFluor® 594 conjugated secondary antibody at 1/500 dilution (red). The nuclear counter stain is DAPI (blue).
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![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [18A5DB1] (ab110328)](https://www.abcam.com/ps/products/110/ab110328/Images/ab110328-123634-anti-frataxin-antibody-18a5db1-immunohistochemistry.jpg)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [18A5DB1] (ab110328)IHC image of Frataxin staining in Human colon adenocarcinoma formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab110328, 10µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
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![Flow Cytometry - Anti-Frataxin antibody [18A5DB1] (ab110328)](https://www.abcam.com/ps/products/110/ab110328/Images/ab110328-123635-anti-frataxin-antibody-18a5db1-flow-cytometry.gif)
Flow Cytometry - Anti-Frataxin antibody [18A5DB1] (ab110328)Flow cytometric analysis using ab110328 at 1µg/ml staining Frataxin in HL60 cells (blue). Isotype control antibody (red). -
![Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [18A5DB1] (ab110328)](https://www.abcam.com/ps/products/110/ab110328/Images/ab110328-123636-anti-frataxin-antibody-18a5db1-immunofluorescence.gif)
Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [18A5DB1] (ab110328)Immunocytochemistry analysis using ab110328 at 0.5µg/ml staining Frataxin in normal Human fibroblasts (MRC5) (fixed and permeabilized) followed by an AlexaFluor® 594-conjugated-goat-anti-mouse IgG1 isotype specific secondary antibody (2 µg/ml). .
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (33)
ab110328 has been referenced in 33 publications.
- Johnson J et al. Drp1-dependent peptide reverse mitochondrial fragmentation, a homeostatic response in Friedreich ataxia. Pharmacol Res Perspect 9:e00755 (2021). PubMed: 33951329
- Picca A et al. Altered Expression of Mitoferrin and Frataxin, Larger Labile Iron Pool and Greater Mitochondrial DNA Damage in the Skeletal Muscle of Older Adults. Cells 9:N/A (2020). PubMed: 33276460
- Agrò M & Díaz-Nido J Effect of Mitochondrial and Cytosolic FXN Isoform Expression on Mitochondrial Dynamics and Metabolism. Int J Mol Sci 21:N/A (2020). PubMed: 33158039
- Tiano F et al. Frataxin deficiency in Friedreich's ataxia is associated with reduced levels of HAX-1, a regulator of cardiomyocyte death and survival. Hum Mol Genet 29:471-482 (2020). PubMed: 31943004
- Rocca CJ et al. CRISPR-Cas9 Gene Editing of Hematopoietic Stem Cells from Patients with Friedreich's Ataxia. Mol Ther Methods Clin Dev 17:1026-1036 (2020). PubMed: 32462051