Mouse Monoclonal Frataxin antibody - conjugated to Alexa Fluor® 488. Suitable for ICC/IF and reacts with Mouse samples.
IgG1
Mouse
Alexa Fluor® 488
Ex: 495nm, Em: 519nm
pH: 7.4
Preservative: 0.02% Sodium azide
Constituents: PBS, 30% Glycerol (glycerin, glycerine), 1% BSA
Liquid
Monoclonal
ICC/IF | |
---|---|
Mouse | Tested |
Species | Dilution info | Notes |
---|---|---|
Species Mouse | Dilution info 1/100 | Notes This product gave a positive signal in NIH3T3 cells fixed with 4% formaldehyde (10 min) and 100% methanol (5 min). |
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Promotes 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. Modulates the RNA-binding activity of ACO1 (By similarity).
Fxn, Fxn, Frda
Mouse Monoclonal Frataxin antibody - conjugated to Alexa Fluor® 488. Suitable for ICC/IF and reacts with Mouse samples.
Fxn, Fxn, Frda
IgG1
Mouse
Alexa Fluor® 488
Ex: 495nm, Em: 519nm
pH: 7.4
Preservative: 0.02% Sodium azide
Constituents: PBS, 30% Glycerol (glycerin, glycerine), 1% BSA
Liquid
Monoclonal
17A11
Affinity purification
kappa
Blue Ice
1-2 weeks
+4°C
-20°C
Upon delivery aliquot
Avoid freeze / thaw cycle, Store in the dark
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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.
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.
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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This species and application combination has not been tested, but we predict it will work based on strong homology. However, this combination is not covered by our product promise.
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ab197963 staining Frataxin in NIH3T3 cells. The cells were fixed with 4% formaldehyde (10 min), permeabilized with 0.1% Triton X-100 for 5 minutes and then blocked with 1% BSA/10% normal goat serum/0.3M glycine in 0.1% PBS-Tween for 1h. The cells were then incubated overnight at +4°C with ab197963 at a 1/100 dilution (shown in green) and Alexa Fluor® 594 Anti-alpha Tubulin antibody [DM1A] - Microtubule Marker ab195889, Mouse monoclonal to alpha Tubulin (Alexa Fluor® 594), at a 1/250 dilution (shown in red). Nuclear DNA was labelled with DAPI (shown in blue).
Image was taken with a confocal microscope (Leica-Microsystems, TCS SP8).
This product also gave a positive signal under the same testing conditions in NIH3T3 cells fixed with 100% methanol (5 min).
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