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The antibody against FXN was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN (NP_000135.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
The antibody against FXN was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN (NP_000135.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
Western blot analysis of various lysates, using FXN Rabbit pAb at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 10s.
| Cat.No | ADA-03933A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | Fxn |
| Target Synonyms | FA; X25; CyaY; FARR; FRDA; FXN | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | 293T, HepG2 | Application | ELISA, WB, IF/ICC, IHC-P |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN (NP_000135.2). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | LRTDIDATCTPRRASSNQRGLNQIWNVKKQSVYLMNLRKSGTLGHPGSLDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLSSLAYSGKDA | Uniprot ID | Q16595 |
Uniprot Id
Q16595
Target Species
Human
Target Name
FXN
Target Full Name
Frataxin, mitochondrial
Target Function
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; 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.
Target Involvement
Friedreich ataxia (FRDA)
Target Subcellular Location
Mitochondrion. Cytoplasm, cytosol.
Target Protein Families
Frataxin family
Target Tissue Specificity
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Target Research Area
Tags & Cell Markers
Target Synonyms
CyaY; d-FXN; FA; FARR; Frataxin mature form; Frataxin(81-210); FRDA; FRDA_HUMAN; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; X25
Target Background
This nuclear gene encodes a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA from 8-33 repeats to >90 repeats results in Friedreich ataxia. Alternative splicing results in multiple transcript variants.
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