Product nameAnti-Frataxin antibody [EPR6107]
See all Frataxin primary antibodies
DescriptionRabbit monoclonal [EPR6107] to Frataxin
Tested applicationsSuitable for: WBmore details
Unsuitable for: IHC-P
Species reactivityReacts with: Human
Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) corresponding to Human Frataxin aa 150 to the C-terminus.
- Human heart, Human fetal skin and PBL lysates.
Mouse and 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.
We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.
This product is a recombinant rabbit monoclonal antibody.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Stable for 12 months at -20°C.
Dissociation constant (KD)KD = 3.19 x 10 -10 M Learn more about KD
Storage bufferpH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 40% Glycerol, 0.05% BSA, 59% PBS
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab124680 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/10000. Detects a band of approximately 17, 30 kDa (predicted molecular weight: 19, 23 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 : Anti-Frataxin antibody [EPR6107] (ab124680) at 1/5000 dilution (purified)
Lane 1 : Human fetal heart lysate
Lane 2 : Human skin lysate
Lysates/proteins at 20 µg per lane.
All lanes : Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/2000 dilution
Predicted band size: 19, 23 kDa
Observed band size: 30 kDa why is the actual band size different from the predicted?
Blocking and diluting buffer 5% NFDM/TBST
All lanes : Anti-Frataxin antibody [EPR6107] (ab124680) at 1/1000 dilution (unpurified)
Lane 1 : Human heart lysate
Lane 2 : Human fetal skin lysate
Lane 3 : PBL lysate
Lysates/proteins at 10 µg per lane.
All lanes : Goat anti-Rabbit HRP at 1/2000 dilution
Predicted band size: 19, 23 kDa
Observed band size: 17,30 kDa why is the actual band size different from the predicted?
Equilibrium disassociation constant (KD)
Learn more about KD
Click here to learn more about KD
ab124680 has not yet been referenced specifically in any publications.