Key features and details
- HRP Mouse monoclonal [17A11] to Frataxin
- Suitable for: WB
- Reacts with: Human
- Conjugation: HRP
- Isotype: IgG1
Product nameHRP Anti-Frataxin antibody [17A11]
See all Frataxin primary antibodies
DescriptionHRP Mouse monoclonal [17A11] to Frataxin
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Cow
Recombinant full length protein corresponding to Frataxin.
- WB: Human heart mitochondrial lysate and HepG2 whole cell lysate.
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Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at +4°C. Avoid freeze / thaw cycle. Store In the Dark.
Storage bufferpH: 7.40
Preservative: 0.1% 10% Proclin 300 Solution
Constituents: 30% Glycerol, 1% BSA, PBS
Concentration information loading...
Light chain typekappa
Our Abpromise guarantee covers the use of ab197964 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/5000. Detects a band of approximately 14 kDa (predicted molecular weight: 14 kDa).|
FunctionPromotes 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 specificityExpressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Involvement in diseaseDefects 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 similaritiesBelongs to the frataxin family.
modificationsProcessed 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 localizationCytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.
- Information by UniProt
- CyaY antibody
- d-FXN antibody
- FA antibody
All lanes : HRP Anti-Frataxin antibody [17A11] (ab197964) at 1/5000 dilution
Lane 1 : Human heart tissue lysate - mitochondrial extract (ab110337)
Lane 2 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate
Lysates/proteins at 20 µg per lane.
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 14 kDa
Observed band size: 14 kDa
Exposure time: 20 minutes
This blot was produced using a 4-12% Bis-tris gel under the MES buffer system. The gel was run at 200V for 35 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% milk before being incubated with ab197964 overnight at 4°C. Antibody binding was visualised using ECL development solution ab133406.
ab197964 has not yet been referenced specifically in any publications.