Product nameAnti-Frataxin antibody
See all Frataxin primary antibodies
DescriptionRabbit polyclonal to Frataxin
Tested applicationsSuitable for: ICC/IF, IHC-P, WBmore details
Species reactivityReacts with: Mouse, Rat, Human
Recombinant full length protein corresponding to Human Frataxin.
Database link: Q16595
- WB: Jurkat, K562, Raji, DU 145 and HL-60 cell lysates; Mouse heart and liver tissue lysates. IHC-P: Mouse and rat heart tissues; ICC/IF: L929 cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol, 49% PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab175402 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use at an assay dependent concentration.|
|IHC-P||1/50 - 1/200.
ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.
|WB||1/500 - 1/2000. Detects a band of approximately 14 kDa (predicted molecular weight: 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 (ab175402) at 1/1000 dilution
Lane 1 : Jurkat (human T cell leukemia cell line from peripheral blood) cell lysate
Lane 2 : K562 (human chronic myelogenous leukemia cell line from bone marrow) cell lysate
Lane 3 : Raji (human Burkitt's lymphoma cell line) cell lysate
Lane 4 : DU 145 (human prostate carcinoma cell line) cell lysate
Lane 5 : HL-60 (human promyelocytic leukemia cell line) cell lysate
Lane 6 : Mouse heart lysate
Lane 7 : Mouse liver lysate
Lysates/proteins at 25 µg per lane.
All lanes : HRP Goat Anti-Rabbit IgG
Predicted band size: 23 kDa
Observed band size: 14 kDa why is the actual band size different from the predicted?
Paraffin-embedded mouse heart tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis.
Immunofluorescence staining of L929 cells stained for Frataxin with ab175402 at 1/100 dilution. DAPI was used as a nuclear counterstain.
Paraffin-embedded rat heart tissue labelling Frataxin using ab175402 at 1/100 dilution in immunohistochemical analysis.
This product has been referenced in:
- Weng L et al. Evaluation of antibodies for western blot analysis of frataxin protein isoforms. J Immunol Methods 474:112629 (2019). Read more (PubMed: 31279523) »
- Britti E et al. Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment. J Cell Mol Med 22:834-848 (2018). IF . Read more (PubMed: 28980774) »