Overview

  • Product name
  • Description
    Rabbit polyclonal to Frataxin
  • 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; MDA-MB453s cell extract; Mouse heart and liver lysates. IHC-P: Human liver cancer tissue; Mouse heart tissue; Rat heart, liver and kidney tissues. ICC/IF: MCF7 and A549 cells.

Properties

Applications

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.
IHC-P 1/50 - 1/200.

ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.

WB 1/500 - 1/2000. Detects a band of approximately 14 kDa (predicted molecular weight: 23 kDa).

Target

  • 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
    modifications
    Processed 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
  • Database links
  • Alternative names
    • CyaY antibody
    • d-FXN antibody
    • FA antibody
    • FARR antibody
    • Frataxin mature form antibody
    • Frataxin(81-210) antibody
    • FRDA antibody
    • FRDA_HUMAN antibody
    • Friedreich ataxia protein antibody
    • Fxn antibody
    • i-FXN antibody
    • m56-FXN antibody
    • m78-FXN antibody
    • m81-FXN antibody
    • MGC57199 antibody
    • X25 antibody
    see all

Images

  • Paraffin-embedded mouse heart tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis. 

  • All lanes : Anti-Frataxin antibody (ab175402)

    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


    Predicted band size : 23 kDa
    Observed band size : 14 kDa (why is the actual band size different from the predicted?)
  • Paraffin-embedded rat heart tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis. 

  • Immunocytochemistry/Immunofluorescence analysis of MCF7 cells using ab175402. Blue DAPI for nuclear staining.
  • Anti-Frataxin antibody (ab175402) at 1/500 dilution + MDA-MB453s cell extract

    Predicted band size : 23 kDa
    Observed band size : 14 kDa (why is the actual band size different from the predicted?)
  • Immunocytochemistry/Immunofluorescence analysis of A549 cells labeling FXN with ab175402 (shown in green) at a dilution of 1/50. Nuclear DNA was labelled with DAPI (shown in blue).
  • Paraffin-embedded rat kidney tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis. Magnification: 200x.

  • Paraffin-embedded human liver cancer tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis. Magnification: 200x.

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of rat liver tissue labelling Frataxin with ab175402.

References

ab175402 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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