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Rabbit Recombinant Monoclonal Frataxin antibody. Suitable for WB and reacts with Human, Recombinant fragment - Human samples. Cited in 1 publication.

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Images

Western blot - Anti-Frataxin antibody [EPR6107] (AB124680), expandable thumbnail
  • OI-RD Scanning - Anti-Frataxin antibody [EPR6107] (AB124680), expandable thumbnail
  • Western blot - Anti-Frataxin antibody [EPR6107] (AB124680), expandable thumbnail
  • Western blot - Anti-Frataxin antibody [EPR6107] (AB124680), expandable thumbnail

Publications

Key facts

Isotype
IgG
Host species
Rabbit
Storage buffer

pH: 7.2 - 7.4
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA

Form
Liquid
Clonality
Monoclonal

Immunogen

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

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Reactivity data

Select an application
Product promiseTestedExpectedPredictedNot recommended
WBIHC-P
Human
Tested
Not recommended
Recombinant fragment - Human
Tested
Not recommended

Tested
Tested

Species
Human
Dilution info
1/1000.00000 - 1/10000.00000
Notes

-

Species
Recombinant fragment - Human
Dilution info
1/1000.00000 - 1/10000.00000
Notes

-

Not recommended
Not recommended

Species
Human, Recombinant fragment - Human
Dilution info
-
Notes

-

Associated Products

Select an associated product type

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Target data

Function

Frataxin mature form. Functions 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 frataxin. Modulates 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

FRDA, X25, FXN, Friedreich ataxia protein, Fxn

Recommended products

Rabbit Recombinant Monoclonal Frataxin antibody. Suitable for WB and reacts with Human, Recombinant fragment - Human samples. Cited in 1 publication.

Key facts

Isotype
IgG
Form
Liquid
Clonality
Monoclonal
Immunogen
  • The exact immunogen used to generate this antibody is proprietary information.
Clone number
EPR6107
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.

Dissociation constant
3.19 x 10-10 M
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
Stable for 12 months at -20°C

Notes

Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

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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4 product images

  • Western blot - Anti-Frataxin antibody [EPR6107] (ab124680), expandable thumbnail

    Western blot - Anti-Frataxin antibody [EPR6107] (ab124680)

    Blocking and diluting buffer 5% NFDM/TBST

    All lanes: Western blot - Anti-Frataxin antibody [EPR6107] (ab124680) at 1/5000 dilution

    Lane 1: Human fetal heart tissue lysate at 20 µg

    Lane 2: Human skin lysate at 20 µg

    Secondary

    All lanes: Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/2000 dilution

    Predicted band size: 23 kDa

    Observed band size: 30 kDa

  • OI-RD Scanning - Anti-Frataxin antibody [EPR6107] (ab124680), expandable thumbnail

    OI-RD Scanning - Anti-Frataxin antibody [EPR6107] (ab124680)

    We have systematically measured KD (the equilibrium dissociation constant between the antibody and its antigen), of more than 840 recombinant antibodies to assess not only their individual KD values but also to see the average affinity of antibody.
    Based on the comparison with published literature values for mouse monoclonal antibodies, Recombinant antibodies appear to be on average 1-2 order of magnitude higher affinity.

  • Western blot - Anti-Frataxin antibody [EPR6107] (ab124680), expandable thumbnail

    Western blot - Anti-Frataxin antibody [EPR6107] (ab124680)

    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.

    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 [EPR6107] (ab124680) at 1/1000 dilution

    Lane 1: Human liver tissue lysate at 20 µg

    Lane 2: Human hippocampus tissue lysate 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

  • Western blot - Anti-Frataxin antibody [EPR6107] (ab124680), expandable thumbnail

    Western blot - Anti-Frataxin antibody [EPR6107] (ab124680)

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

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

    All lanes: Western blot - Anti-Frataxin antibody [EPR6107] (ab124680) 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: 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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