• Immunofluorescence analysis of A-431 cells using FXN Rabbit polyclonal antibody (STJ11101185) at a dilution of 1:200 (40x lens). Secondary antibody: Cy3 Goat Anti-Rabbit IgG (H+L) at 1:500 dilution. Blue: DAPI for nuclear staining.
  • Immunofluorescence analysis of Hep G2 cells using FXN Rabbit polyclonal antibody (STJ11101185) at a dilution of 1:200 (40x lens). Secondary antibody: Cy3 Goat Anti-Rabbit IgG (H+L) at 1:500 dilution. Blue: DAPI for nuclear staining.
  • Immunohistochemistry analysis of FXN in paraffin-embedded rat pancreas using FXN Rabbit polyclonal antibody (STJ11101185) at dilution of 1:100 (40x lens). Perform microwave antigen retrieval with 10 mM PBS buffer pH 7. 2 before commencing with immunohistochemistry staining protocol.
  • Immunohistochemistry analysis of FXN in paraffin-embedded rat lung using FXN Rabbit polyclonal antibody (STJ11101185) at dilution of 1:100 (40x lens). Perform microwave antigen retrieval with 10 mM PBS buffer pH 7. 2 before commencing with immunohistochemistry staining protocol.
  • Western blot analysis of various lysates, using FXN Rabbit polyclonal antibody (STJ11101185) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (STJS000856) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 10s.

Anti-FXN antibody (42-210) (STJ11101185)

SKU:
STJ11101185

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Host: Rabbit
Applications: WB/IHC/IF
Reactivity: Human/Mouse/Rat
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Short Description: Rabbit polyclonal antibody anti-FXN (42-210) is suitable for use in Western Blot, Immunohistochemistry and Immunofluorescence research applications.
Clonality: Polyclonal
Conjugation: Unconjugated
Isotype: IgG
Formulation: PBS with 0.01% Thimerosal, 50% Glycerol, pH7.3.
Purification: Affinity purification
Dilution Range: WB 1:500-1:2000
IHC-P 1:50-1:200
IF/ICC 1:50-1:200
Storage Instruction: Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles.
Gene Symbol: FXN
Gene ID: 2395
Uniprot ID: FRDA_HUMAN
Immunogen Region: 42-210
Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN (NP_000135.2).
Immunogen Sequence: LRTDIDATCTPRRASSNQRG LNQIWNVKKQSVYLMNLRKS GTLGHPGSLDETTYERLAEE TLDSLAEFFEDLADKPYTFE DYDVSFGSGVLTVKLGGDLG TYVINKQTPNKQIWLSSPSS GPKRYDWTGKNWVYSHDGVS LHELLAAELTKALKTKLDLS SLAYSGKDA
Tissue Specificity Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Post Translational Modifications Processed 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.
Function Frataxin mature form: Functions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the 2Fe-2S cluster assembly. Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release. Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during 2Fe-2S cluster assembly. The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a 2Fe-2S cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the 2Fe-2S cluster assembly. Finally, the 2Fe-2S cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5. 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. May function as an iron chaperone protein that protects the aconitase 4Fe-4S2+ cluster from disassembly and promotes enzyme reactivation. May play a role as a high affinity iron binding partner for FECH that is capable of both delivering iron to ferrochelatase and mediating the terminal step in mitochondrial heme biosynthesis. Extramitochondrial frataxin: Modulates the RNA-binding activity of ACO1. May be involved in the cytoplasmic iron-sulfur protein biogenesis. May contribute to oxidative stress resistance and overall cell survival.
Protein Name Frataxin - Mitochondrial
Friedreich Ataxia Protein
Fxn Cleaved Into - Frataxin Intermediate Form
I-Fxn - Frataxin(56-210
M56-Fxn - Frataxin(78-210
D-Fxn
M78-Fxn - Frataxin Mature Form
Frataxin(81-210
M81-Fxn - Extramitochondrial Frataxin
Database Links Reactome: R-HSA-1268020
Reactome: R-HSA-1362409
Cellular Localisation Frataxin Mature Form: Mitochondrion
Extramitochondrial Frataxin: Cytoplasm
Cytosol
Alternative Antibody Names Anti-Frataxin - Mitochondrial antibody
Anti-Friedreich Ataxia Protein antibody
Anti-Fxn Cleaved Into - Frataxin Intermediate Form antibody
Anti-I-Fxn - Frataxin(56-210 antibody
Anti-M56-Fxn - Frataxin(78-210 antibody
Anti-D-Fxn antibody
Anti-M78-Fxn - Frataxin Mature Form antibody
Anti-Frataxin(81-210 antibody
Anti-M81-Fxn - Extramitochondrial Frataxin antibody
Anti-FXN antibody
Anti-FRDA antibody
Anti-X25 antibody

Information sourced from Uniprot.org

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