AB244232

Anti-Frataxin antibody [EPR6107] - BSA and Azide free

RabMAb
Recombinant
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(1 Publication)

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

View Alternative Names

FRDA, X25, FXN, Friedreich ataxia protein, Fxn

4 Images

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Western blot - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

This data was developed using ab124680, the same antibody clone in a different buffer formulation.

Blocking and dilution buffer : 5% NFDM/TBST.

All lanes:

Western blot - Anti-Frataxin antibody [EPR6107] (<a href='/en-us/products/primary-antibodies/frataxin-antibody-epr6107-ab124680'>ab124680</a>) 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

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Supplier Data

Western blot - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

This data was developed using ab124680, the same antibody clone in a different buffer formulation.

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.

ab181602 was used as a loading control.

All lanes:

Western blot - Anti-Frataxin antibody [EPR6107] (<a href='/en-us/products/primary-antibodies/frataxin-antibody-epr6107-ab124680'>ab124680</a>) 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) (<a href='/en-us/products/secondary-antibodies/goat-rabbit-igg-h-l-hrp-ab97051'>ab97051</a>) at 1/20000 dilution

Predicted band size: 23 kDa

Observed band size: 14 kDa,17 kDa

true

Exposure time: 60s

Lab

Western blot - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

This data was developed using ab124680, the same antibody clone in a different buffer formulation. Blocking and diluting buffer and concentration : 5% NFDM/TBST. ab213204 was used as a loading control.

All lanes:

Western blot - Anti-Frataxin antibody [EPR6107] (<a href='/en-us/products/primary-antibodies/frataxin-antibody-epr6107-ab124680'>ab124680</a>) 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) (<a href='/en-us/products/secondary-antibodies/goat-rabbit-igg-h-l-hrp-ab97051'>ab97051</a>) at 1/20000 dilution

Predicted band size: 23 kDa

Observed band size: 16 kDa

false

Exposure time: 60s

OI-RD Scanning - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

OI-RD Scanning

Unknown

OI-RD Scanning - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

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.

Unconjugated

Anti-Frataxin antibody [EPR6107]

Key facts

Host species

Rabbit

Clonality

Monoclonal

Clone number

EPR6107

Isotype

IgG

Carrier free

Yes

Reacts with

Human

Applications

applications

Immunogen

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

Specificity

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

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Recombinant fragment - Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" } } }

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Product details

ab244232 is the carrier-free version of ab124680.

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

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

What are the advantages of a recombinant monoclonal antibody?
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.

Conjugation ready
Our carrier-free antibodies are typically supplied in a PBS-only formulation, purified and free of BSA, sodium azide and glycerol. This conjugation-ready format is designed for use with fluorochromes, metal isotopes, oligonucleotides, and enzymes, which makes them ideal for antibody labelling, functional and cell-based assays, flow-based assays (e.g. mass cytometry) and Multiplex Imaging applications.

Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with 1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.

Compatibility
This product is compatible with the Maxpar^® Antibody Labeling Kit from Fluidigm, without the need for antibody preparation. Maxpar^® is a trademark of Fluidigm Canada Inc.

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Properties and storage information

Form

Liquid

Purification technique

Affinity purification Protein A

Storage buffer

pH: 7.2 - 7.4 Constituents: PBS

Shipped at conditions

Blue Ice

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

+4°C

Storage information

Do Not Freeze

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Supplementary information

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

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.

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.

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Product protocols

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

Visit the General protocols
Visit the Troubleshooting

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Target data

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).

See full target information FXN

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Publications (1)

Recent publications for all applications. Explore the full list and refine your search

iScience 28:112992 PubMed40687785

2025

Differential substrate degradation by super-degron: EGFP in wild-type mouse cells, PD-1 requires CRBN humanization.

Applications

Unspecified application

Species

Unspecified reactive species

Chie Naruse,Ojiro Ishibashi,Masatoshi Ohgushi,Hirohiko Imai,Tomoko Matsuzaki,Xuchi Pan,Tatsuhiko Miyazaki,Yuka Shidahara,Yu Shirakawa,Fumihiro Sugiyama,Masahide Asano

View all publications

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Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.

For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

For licensing inquiries, please contact partnerships@abcam.com

Anti-Frataxin antibody [EPR6107] - BSA and Azide free

Western blot - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

Western blot - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

Western blot - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

OI-RD Scanning - Anti-Frataxin antibody [EPR6107] - BSA and Azide free (AB244232)

Related conjugates and formulations (1)

Key facts

Host species

Clonality

Clone number

Isotype

Carrier free

Reacts with

Applications

Immunogen

Specificity

Alternative Products

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Assay Kits

Secondary Antibodies

Primary Antibodies

Primary Antibodies

Primary Antibodies

Reactivity data

Product details

Properties and storage information

Form

Purification technique

Storage buffer

Shipped at conditions

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Storage information

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Publications (1)

Product promise