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Rabbit Recombinant Monoclonal Frataxin antibody. Suitable for IP, WB, ICC/IF, Flow Cyt (Intra), IHC-P and reacts with Mouse, Human, Rat, Recombinant fragment - Human samples. Cited in 3 publications.

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Images

Western blot - Anti-Frataxin antibody [EPR21840] (AB219414), expandable thumbnail
  • Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (AB219414), expandable thumbnail
  • Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (AB219414), expandable thumbnail
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (AB219414), expandable thumbnail
  • Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [EPR21840] (AB219414), expandable thumbnail

Publications

Key facts

Isotype

IgG

Host species

Rabbit

Storage buffer

pH: 7.2 - 7.4
Preservative: 0.01% Sodium azide
Constituents: PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA

Form

Liquid

Clonality

Monoclonal

Immunogen

  • The exact immunogen used to generate this antibody is proprietary information.

Reactivity data

Select an application
Product promiseTestedExpectedPredictedNot recommended
IPWBICC/IFFlow Cyt (Intra)IHC-P
Human
Tested
Tested
Tested
Tested
Tested
Mouse
Tested
Tested
Tested
Expected
Tested
Rat
Expected
Tested
Expected
Expected
Tested
Recombinant fragment - Human
Not recommended
Tested
Not recommended
Not recommended
Not recommended

Tested
Tested

Species

Mouse

Dilution info

1/30

Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Species

Human

Dilution info

1/30

Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Expected
Expected

Species

Rat

Dilution info

Use at an assay dependent concentration.

Notes

-

Not recommended
Not recommended

Species

Recombinant fragment - Human

Dilution info

-

Notes

-

Tested
Tested

Species

Mouse

Dilution info

1/1000

Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Species

Rat

Dilution info

1/1000

Notes

-

Species

Human

Dilution info

1/1000

Notes

-

Species

Recombinant fragment - Human

Dilution info

-

Notes

-

Tested
Tested

Species

Mouse

Dilution info

1/50

Notes

-

Species

Human

Dilution info

1/50

Notes

-

Expected
Expected

Species

Rat

Dilution info

Use at an assay dependent concentration.

Notes

-

Not recommended
Not recommended

Species

Recombinant fragment - Human

Dilution info

-

Notes

-

Tested
Tested

Species

Human

Dilution info

1/60

Notes

-

Expected
Expected

Species

Mouse, Rat

Dilution info

Use at an assay dependent concentration.

Notes

-

Not recommended
Not recommended

Species

Recombinant fragment - Human

Dilution info

-

Notes

-

Tested
Tested

Species

Mouse

Dilution info

1/500

Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Species

Rat

Dilution info

1/500

Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Species

Human

Dilution info

1/500

Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Not recommended
Not recommended

Species

Recombinant fragment - Human

Dilution info

-

Notes

-

Associated Products

Select an associated product type

9 products for Alternative Product

Target data

Function

Frataxin mature formFunctions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the [2Fe-2S] cluster assembly (PubMed:12785837, PubMed:24971490). Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release (PubMed:24971490). Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly (PubMed:29576242). The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly. Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (By similarity). 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 (PubMed:15641778). 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 (PubMed:11823441, PubMed:12755598). May function as an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation (PubMed:15247478). May play a role as a high affinity iron binding partner for FECH that is capable of both delivering iron to ferrochelatase and mediating the terminal step in mitochondrial heme biosynthesis (PubMed:15123683, PubMed:16239244).Extramitochondrial frataxinModulates the RNA-binding activity of ACO1 (PubMed:20053667). May be involved in the cytoplasmic iron-sulfur protein biogenesis (PubMed:16091420). May contribute to oxidative stress resistance and overall cell survival (PubMed:16608849).

Alternative names

Recommended products

Rabbit Recombinant Monoclonal Frataxin antibody. Suitable for IP, WB, ICC/IF, Flow Cyt (Intra), IHC-P and reacts with Mouse, Human, Rat, Recombinant fragment - Human samples. Cited in 3 publications.

Key facts

Isotype

IgG

Form

Liquid

Clonality

Monoclonal

Immunogen
  • The exact immunogen used to generate this antibody is proprietary information.
Clone number

EPR21840

Purification technique

Affinity purification Protein A

Specificity

We suggest optimizing experimental protocols (increasing lysate amount, using lower dilution or higher sensitivity ECL substrate) to improve results.

Concentration
Loading...

Storage

Shipped at conditions

Blue Ice

Appropriate short-term storage duration

1-2 weeks

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

-20°C

Aliquoting information

Upon delivery aliquot

Storage information

Avoid freeze / thaw cycle

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 monoclonal antibody, which offers several advantages including:

  • - High batch-to-batch consistency and reproducibility
  • - Improved sensitivity and specificity
  • - Long-term security of supply
  • - Animal-free batch production

For more information, read more on recombinant antibodies.

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Frataxin often known by the alternate name FXN is a mitochondrial protein with a mass of approximately 21000 Dalton. It is expressed mainly in tissues with high energy demands like the heart liver and pancreas. Frataxin plays an important role in iron-sulfur cluster assembly which is essential for various cellular processes. The protein is a part of mitochondria where it regulates iron homeostasis and prevents oxidative damage by minimizing iron-induced free radical generation.

Biological function summary

Several cellular processes depend on the correct function of this protein. Frataxin assists in forming iron-sulfur clusters acting within a multiprotein complex in the mitochondria. The complex includes proteins such as ISCU which are involved in the assembly and repair of iron-sulfur clusters. These clusters are necessary for supporting mitochondrial electron transport and other fundamental metabolic pathways that require iron-sulfur dependencies.

Pathways

Frataxin's involvement extensively affects the mitochondrial respiratory chain and the mitochondrial biogenesis process. It plays a role in the electron transport chain by stabilizing iron-sulfur-containing complexes. NAB is one associated protein that interacts closely within these pathways sharing a connection through iron-sulfur cluster transportation and assembly systems. Efficient function of these pathways ensures a proper energetic output of cells.

Associated diseases and disorders

Frataxin mutations are directly linked to Friedreich's ataxia a neurodegenerative disease causing progressive damage to the nervous system. The deficiency or dysfunction in frataxin causes accumulation of iron in mitochondria leading to increased oxidative stress. Another related disorder includes heart disease which emerges due to the same oxidative stress pathway. Proteins such as Nfs1 are also involved sharing the responsibility with frataxin in scavenging excess iron protecting against related tissue damage.

Product promise

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12 product images

  • Western blot - Anti-Frataxin antibody [EPR21840] (ab219414), expandable thumbnail

    Western blot - Anti-Frataxin antibody [EPR21840] (ab219414)

    Blockingand dilution buffer: 5% NFDM/TBST .

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

    Lane 1: HCT 116 (human colorectal carcinoma cell line) whole cell lysate at 10 µg

    Lane 2: Neuro-2a (mouse neuroblastoma cell line) whole cell lysate at 10 µg

    Lane 3: C6 (rat glial tumor glial cell) whole cell lysate at 10 µg

    Lane 4: PC-12 (rat adrenal gland pheochromocytoma cell line) whole cell lysate at 10 µg

    Secondary

    Lanes 1 - 2: Western blot - Goat Anti-Rabbit IgG H&L (HRP) (Goat Anti-Rabbit IgG H&L (HRP) ab97051) at 1/10000 dilution

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

    Predicted band size: 23 kDa

    Observed band size: 14 kDa

    Exposure time: 3min

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

    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) (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 (Rabbit IgG, monoclonal [EPR25A] - Isotype Control ab172730) instead of ab219414 in Neuro-2a whole cell lysate (-).


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

    All lanes: Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (ab219414)

    Predicted band size: 23 kDa

    Observed band size: 14 kDa

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

    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) (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 (Rabbit IgG, monoclonal [EPR25A] - Isotype Control ab172730) instead of ab219414 in HCT 116 whole cell lysate (-).


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

    All lanes: Immunoprecipitation - Anti-Frataxin antibody [EPR21840] (ab219414)

    Predicted band size: 13 kDa, 23 kDa

    Observed band size: 17 kDa

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

    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 Antigen Retrieval Buffer (100X Tris-EDTA Buffer, pH 9.0) ab93684 (Tris/EDTA buffer pH 9.0).

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

    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) (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 Anti-COX IV antibody [mAbcam33985] - Mitochondrial Marker ab33985 Anti-COX IV antibody - Mitochondrial Marker at a 1/1000 dilution followed by Goat Anti-Mouse IgG H&L (Alexa Fluor® 594) (Goat Anti-Mouse IgG H&L (Alexa Fluor® 594) preadsorbed ab150120) (orange). The nuclear counter stain is DAPI (blue).

    The negative control is the secondary antibody only.

  • Flow Cytometry (Intracellular) - Anti-Frataxin antibody [EPR21840] (ab219414), expandable thumbnail

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

    Intracellular 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 (Rabbit IgG, monoclonal [EPR25A] - Isotype Control ab172730) (black) and an unlabeled control (cells without incubation with primary antibody and secondary antibody) (blue). Goat anti rabbit IgG (Alexa Fluor® 488, Goat Anti-Rabbit IgG H&L (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), expandable thumbnail

    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 Antigen Retrieval Buffer (100X Tris-EDTA Buffer, pH 9.0) ab93684 (Tris/EDTA buffer pH 9.0).

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

    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 Antigen Retrieval Buffer (100X Tris-EDTA Buffer, pH 9.0) ab93684 (Tris/EDTA buffer pH 9.0).

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

    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 Antigen Retrieval Buffer (100X Tris-EDTA Buffer, pH 9.0) ab93684 (Tris/EDTA buffer pH 9.0).

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

    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) (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 Alexa Fluor® 594 Anti-alpha Tubulin antibody [DM1A] - Microtubule Marker 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.

  • Western blot - Anti-Frataxin antibody [EPR21840] (ab219414), expandable thumbnail

    Western blot - Anti-Frataxin antibody [EPR21840] (ab219414)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST.

    Anti-6X His tag® antibody [EPR20547] - ChIP Grade ab213204 was used as laoding control.

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

    All lanes: His tagged Human FXN recombinant protein (aa 82-210) at 10 ng

    Secondary

    All lanes: Western blot - Goat Anti-Rabbit IgG H&L (HRP) (Goat Anti-Rabbit IgG H&L (HRP) ab97051) at 1/20000 dilution

    Predicted band size: 23 kDa

    Observed band size: 16 kDa

    Exposure time: 3s

  • Western blot - Anti-Frataxin antibody [EPR21840] (ab219414), expandable thumbnail

    Western blot - Anti-Frataxin antibody [EPR21840] (ab219414)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST.

    This blot was developed using a high sensitivity ECL substrate. The high-sensitivity ECL substrate used allows for the detection of proteins in the mid-femtogram range.

    ab219414 is not suitable for testing precursor form, we recommend Anti-Frataxin antibody [EPR6107] ab124680 as an alternative for precursor form testing.

    For different forms of frataxin, you can refer to PMID: 17468497, PMID: 31279523, PMID: 17468497 etc.

    Anti-GAPDH antibody [EPR16891] - Loading Control ab181602 was used as a loading control.

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

    Lane 1: Human liver tissue lysate at 20 µg

    Lane 2: Human hippocampus tissue at 20 µg

    Lane 3: HCT116 (human colorectal carcinoma epithelial cell) whole cell lysate at 20 µg

    Lane 4: Huh7 (human hepatocellular carcinoma epithelial cell) whole cell lysate at 20 µg

    Lane 5: SH-SY5Y (human neuroblastoma epithelial cell) whole cell lysate at 20 µg

    Secondary

    All lanes: Western blot - Goat Anti-Rabbit IgG H&L (HRP) (Goat Anti-Rabbit IgG H&L (HRP) ab97051) at 1/20000 dilution

    Developed using the ECL technique.

    Predicted band size: 23 kDa

    Observed band size: 14,17 kDa

    Exposure time: 60s

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can:

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