Anti-Frataxin antibody (ab175402)
Key features and details
- Rabbit polyclonal to Frataxin
- Suitable for: ICC/IF, IHC-P, WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
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Overview
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Product name
Anti-Frataxin antibody
See all Frataxin primary antibodies -
Description
Rabbit polyclonal to Frataxin -
Host species
Rabbit -
Tested applications
Suitable for: ICC/IF, IHC-P, WBmore details -
Species reactivity
Reacts with: Mouse, Rat, Human -
Immunogen
Recombinant full length protein corresponding to Human Frataxin.
Database link: Q16595 -
Positive control
- WB: Jurkat, K562, Raji, DU 145 and HL-60 cell lysates; Mouse heart and liver tissue lysates. IHC-P: Mouse and rat heart tissues; ICC/IF: L929 cells.
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General notes
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
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol, 49% PBS -
Concentration information loading... -
Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab175402 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 |
|---|---|---|
| ICC/IF |
Use at an assay dependent concentration.
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| IHC-P |
1/50 - 1/200.
ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody. |
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| WB |
1/500 - 1/2000. Detects a band of approximately 14 kDa (predicted molecular weight: 23 kDa).
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| Notes |
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ICC/IF
Use at an assay dependent concentration. |
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IHC-P
1/50 - 1/200. ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody. |
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WB
1/500 - 1/2000. Detects a band of approximately 14 kDa (predicted molecular weight: 23 kDa). |
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
- Entrez Gene: 14297 Mouse
- Entrez Gene: 499335 Rat
- Omim: 606829 Human
- SwissProt: Q16595 Human
- SwissProt: O35943 Mouse
- SwissProt: D3ZYW7 Rat
- Unigene: 20685 Human
see all -
Alternative names
- CyaY antibody
- d-FXN antibody
- FA antibody
see all
Images
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Western blot - Anti-Frataxin antibody (ab175402)All lanes : Anti-Frataxin antibody (ab175402) at 1/1000 dilution
Lane 1 : Jurkat (human T cell leukemia cell line from peripheral blood) cell lysate
Lane 2 : K562 (human chronic myelogenous leukemia cell line from bone marrow) cell lysate
Lane 3 : Raji (human Burkitt's lymphoma cell line) cell lysate
Lane 4 : DU 145 (human prostate carcinoma cell line) cell lysate
Lane 5 : HL-60 (human promyelocytic leukemia cell line) cell lysate
Lane 6 : Mouse heart lysate
Lane 7 : Mouse liver lysate
Lysates/proteins at 25 µg per lane.
Secondary
All lanes : HRP Goat Anti-Rabbit IgG
Predicted band size: 23 kDa
Observed band size: 14 kDa why is the actual band size different from the predicted? -
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody (ab175402)Paraffin-embedded mouse heart tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis.
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Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody (ab175402)Immunofluorescence staining of L929 cells stained for Frataxin with ab175402 at 1/100 dilution. DAPI was used as a nuclear counterstain.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody (ab175402)Paraffin-embedded rat heart tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis.
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (9)
ab175402 has been referenced in 9 publications.
- Furihata T et al. Cardiac-specific loss of mitoNEET expression is linked with age-related heart failure. Commun Biol 4:138 (2021). PubMed: 33514783
- Weng L et al. Extra-mitochondrial mouse frataxin and its implications for mouse models of Friedreich's ataxia. Sci Rep 10:15788 (2020). PubMed: 32978498
- Pérez MJ et al. Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer's disease-like pathology in human cerebral organoids. Mol Psychiatry N/A:N/A (2020). PubMed: 32632204
- Weng L et al. Evaluation of antibodies for western blot analysis of frataxin protein isoforms. J Immunol Methods 474:112629 (2019). PubMed: 31279523
- Britti E et al. Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment. J Cell Mol Med 22:834-848 (2018). IF . PubMed: 28980774
- Guo L et al. Characterization of a new N-terminally acetylated extra-mitochondrial isoform of frataxin in human erythrocytes. Sci Rep 8:17043 (2018). PubMed: 30451920
- Mon EE et al. Regulation of mitochondrial iron homeostasis by sideroflexin 2. J Physiol Sci N/A:N/A (2018). PubMed: 30570704
- Lin H et al. Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia. Dis Model Mech 10:1343-1352 (2017). PubMed: 29125827
- Lin H et al. Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia. Dis Model Mech 10:1529-1538 (2017). PubMed: 29259026