Recombinant
RabMAb

Recombinant Anti-Frataxin antibody [EPR21840] (ab219414)

Rabbit recombinant monoclonal Frataxin antibody [EPR21840]. Validated in WB, IP, IHC, Flow Cyt, ICC/IF and tested in Mouse, Rat, Human. Immunogen corresponding to recombinant fragment.

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Overview

  • Product name

    Anti-Frataxin antibody [EPR21840]
    See all Frataxin primary antibodies

  • Description

    Rabbit monoclonal [EPR21840] to Frataxin

  • Host species

    Rabbit

  • Tested applications

    Suitable for: WB, IHC-P, IP, Flow Cyt, ICC/IFmore details

  • Species reactivity

    Reacts with: Mouse, Rat, Human

  • Immunogen

    Recombinant fragment within Human Frataxin aa 50 to the C-terminus. The exact sequence is proprietary.
    Database link: Q16595

  • Positive control

    • WB: HCT 116, Neuro-2a, C6 and PC-12 whole cell lysates. IHC-P: Human testis and liver tissue; Mouse and rat kidney tissue. ICC/IF: HCT 116 and Neuro-2a cells. Flow Cyt: HCT 116 cells. IP: HCT 116 and Neuro-2a cells.

  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab219414 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 1/1000. Detects a band of approximately 14 kDa (predicted molecular weight: 23 kDa).
IHC-P 1/500. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.
IP 1/30.
Flow Cyt 1/60.
ICC/IF 1/50.

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

    see all

  • 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

  • Western blot - Anti-Frataxin antibody [EPR21840] (ab219414)
    Western blot - Anti-Frataxin antibody [EPR21840] (ab219414)
    All lanes : Anti-Frataxin antibody [EPR21840] (ab219414) at 1/1000 dilution

    Lane 1 : HCT 116 (human colorectal carcinoma cell line) whole cell lysate
    Lane 2 : Neuro-2a (mouse neuroblastoma cell line) whole cell lysate
    Lane 3 : C6 (rat glial tumor glial cell) whole cell lysate
    Lane 4 : PC-12 (rat adrenal gland pheochromocytoma cell line) whole cell lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    Lanes 1-2 : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/10000 dilution
    Lanes 3-4 : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution

    Predicted band size: 23 kDa
    Observed band size: 14 kDa
    why is the actual band size different from the predicted?


    Exposure time: 3 minutes


    Blockingand dilution buffer: 5% NFDM/TBST .

     

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)

    Immunohistochemical analysis of paraffin-embedded human testis tissue labeling Frataxin with ab219414 at 1/500 dilution, followed by a ready to use Goat Anti-Rabbit IgG H&L (HRP). Granular cytoplasmic staining in human testis (PMID: 18725397; PMID: 26035392) is observed. Counter stained with Hematoxylin.
    Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is a ready to use Goat Anti-Rabbit IgG H&L (HRP).

    Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer pH 9.0).

  • Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [EPR21840] (ab219414)

    Immunofluorescent analysis of 4% paraformaldehyde-fixed, 0.1% Triton X-100 permeabilized HCT 116 (human colorectal carcinoma epithelial cell) cells labeling Frataxin with ab219414 at 1/50 dilution, followed by Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077) secondary antibody at 1/1000 dilution (green).

    Confocal image showing mitochondrial staining in HCT 116 cell line.

    Counterstained with ab33985 Anti-COX IV antibody - Mitochondrial Marker at a 1/1000 dilution followed by Goat Anti-Mouse IgG H&L (Alexa Fluor® 594) (ab150120) (orange). The nuclear counter stain is DAPI (blue).

    The negative control is the secondary antibody only.

  • Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (ab219414)

    Frataxin was immunoprecipitated from 0.35 mg Neuro-2a (mouse neuroblastoma cell line) whole cell lysate with ab219414 at 1/30 dilution. Western blot was performed from the immunoprecipitate using ab219414 at 1/500 dilution. VeriBlot for IP Detection Reagent (HRP) (ab131366), was used for detection at 1/1000 dilution.


    Lane 1: Neuro-2a whole cell lysate 10 µg (Input).
    Lane 2: ab219414 IP in Neuro-2a whole cell lysate (+).
    Lane 3: Rabbit monoclonal IgG (ab172730) instead of ab219414 in Neuro-2a whole cell lysate (-).


    Blocking/Dilution buffer and concentration: 5% NFDM/TBST.
    Exposure time: 30 seconds.

  • Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (ab219414)

    Frataxin was immunoprecipitated from 0.35 mg HCT 116 (human colorectal carcinoma epithelial cell) whole cell lysate with ab219414 at 1/30 dilution. Western blot was performed from the immunoprecipitate using ab219414 at 1/500 dilution. VeriBlot for IP Detection Reagent (HRP) (ab131366), was used for detection at 1/1000 dilution.


    Lane 1: HCT 116 whole cell lysate 10 µg (Input).
    Lane 2: ab219414 IP in HCT 116 whole cell lysate (+).
    Lane 3: Rabbit monoclonal IgG (ab172730) instead of ab219414 in HCT 116 whole cell lysate (-).


    Blocking/Dilution buffer and concentration: 5% NFDM/TBST.
    Exposure time: 5 seconds.

  • Flow Cytometry - Anti-Frataxin antibody [EPR21840] (ab219414)
    Flow Cytometry - Anti-Frataxin antibody [EPR21840] (ab219414)

    Flow cytometric analysis of 4% paraformaldehyde-fixed, 90% methanol-permeabilized HCT 116 (human colorectal carcinoma epithelial cell) cell line labeling Frataxin with ab219414 at 1/60 (red) compared with a rabbit monoclonal IgG (ab172730) (black) and an unlabeled control (cells without incubation with primary antibody and secondary antibody) (blue). Goat anti rabbit IgG (Alexa Fluor® 488, ab150077), at 1/2000 dilution was used as the secondary antibody.

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)

    Immunohistochemical analysis of paraffin-embedded rat kidney tissue labeling Frataxin with ab219414 at 1/500 dilution, followed by a ready to use Goat Anti-Rabbit IgG H&L (HRP). Granular cytoplasmic staining in rat kidney (PMID: 18725397; PMID: 26035392) is observed. Counter stained with Hematoxylin.
    Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is a ready to use Goat Anti-Rabbit IgG H&L (HRP).

    Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer pH 9.0).

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)

    Immunohistochemical analysis of paraffin-embedded mouse kidney tissue labeling Frataxin with ab219414 at 1/500 dilution, followed by a ready to use Goat Anti-Rabbit IgG H&L (HRP). Granular cytoplasmic staining in mouse kidney (PMID: 18725397; PMID: 26035392) is observed. Counter stained with Hematoxylin.
    Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is a ready to use Goat Anti-Rabbit IgG H&L (HRP).

    Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer pH 9.0).

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (ab219414)

    Immunohistochemical analysis of paraffin-embedded human liver tissue labeling Frataxin with ab219414 at 1/500 dilution, followed by a ready to use Goat Anti-Rabbit IgG H&L (HRP). Granular cytoplasmic staining in human liver (PMID: 18725397; PMID: 26035392) is observed. Counter stained with Hematoxylin.
    Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is a ready to use Goat Anti-Rabbit IgG H&L (HRP).

    Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer pH 9.0).

  • Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [EPR21840] (ab219414)
    Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [EPR21840] (ab219414)

    Immunofluorescent analysis of 4% paraformaldehyde-fixed, 0.1% Triton X-100 permeabilized Neuro-2a (mouse neuroblastoma cell line) cells labeling Frataxin with ab219414 at 1/50 dilution, followed by Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077) secondary antibody at 1/1000 dilution (green).

    Confocal image showing cytoplasmic staining in the Neuro-2a cell line.

    Counterstained with ab195889 Anti-alpha Tubulin antibody [DM1A] - Microtubule Marker (Alexa Fluor® 594) at a 1/200 dilution (red). The nuclear counter stain is DAPI (blue).

    The negative control is the secondary antibody only.

References

ab219414 has not yet been referenced specifically in any publications.

Publishing research using ab219414? Please let us know so that we can cite the reference in this datasheet.

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