Key features and details
- Alexa Fluor® 488 Mouse monoclonal [18A5DB1] to Frataxin
- Suitable for: Flow Cyt
- Reacts with: Human
- Conjugation: Alexa Fluor® 488. Ex: 495nm, Em: 519nm
- Isotype: IgG1
Product nameAlexa Fluor® 488 Anti-Frataxin antibody [18A5DB1]
See all Frataxin primary antibodies
DescriptionAlexa Fluor® 488 Mouse monoclonal [18A5DB1] to Frataxin
ConjugationAlexa Fluor® 488. Ex: 495nm, Em: 519nm
Tested applicationsSuitable for: Flow Cytmore details
Species reactivityReacts with: Human
Does not react with: Mouse, Rat, Cow
Recombinant full length protein corresponding to Human Frataxin aa 50-250.
- Flow Cyt: HepG2 cells.
Product was previously marketed under the MitoSciences sub-brand.
Alexa Fluor® is a registered trademark of Molecular Probes, Inc, a Thermo Fisher Scientific Company. The Alexa Fluor® dye included in this product is provided under an intellectual property license from Life Technologies Corporation. As this product contains the Alexa Fluor® dye, the purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). As this product contains the Alexa Fluor® dye the sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: in manufacturing; (ii) to provide a service, information, or data in return for payment (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are sold for use in research. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or firstname.lastname@example.org.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle. Store In the Dark.
Storage bufferPreservative: 0.02% Sodium azide
Constituents: 30% Glycerol, 1% BSA, 68% PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab156033 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
ab171463 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
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
Overlay histogram showing HepG2 cells stained with ab156033 (red line). The cells were fixed with 4% formaldehyde (10 min) and then permeabilized with 0.1% PBS-Triton X-100 for 15 min. The cells were then incubated in 1x PBS / 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (ab156033, 1/50 dilution) for 30 min at 22°C.
Isotype control antibody (black line) was Mouse IgG1 - Alexa Fluor® 488 (ab171463) used at the same concentration and conditions as the primary antibody. Unlabelled sample (blue line) was also used as a control.
Acquisition of >5,000 events were collected using a 50 mW Blue laser (488nm) and 530/30 bandpass filter.
Flow cytometric analysis of Human lymphocytes labeling Frataxin with ab156033 at 0.2 µg/ml.
Lymphocytes were isolated from Control Patients (red), Freidrich Ataxia Carrier Patients (blue), and Friedrich Ataxia Patients (yellow).
IgG1 Alexa®488 conjugated Isotype control antibody (black at 0.2 µg/ml).
ab156033 has not yet been referenced specifically in any publications.