| Host: |
Rabbit |
| Applications: |
WB/IHC/IF |
| Reactivity: |
Human/Mouse |
| 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/Frataxin (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 1:500-1:1000IHC-P 1:50-1:200IF/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/Frataxin (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 - MitochondrialFriedreich Ataxia ProteinFxn Cleaved Into - Frataxin Intermediate FormI-Fxn - Frataxin(56-210M56-Fxn - Frataxin(78-210D-FxnM78-Fxn - Frataxin Mature FormFrataxin(81-210M81-Fxn - Extramitochondrial Frataxin |
| Database Links | Reactome: R-HSA-1268020Reactome: R-HSA-1362409 |
| Cellular Localisation | Frataxin Mature Form: MitochondrionExtramitochondrial Frataxin: CytoplasmCytosol |
| Alternative Antibody Names | Anti-Frataxin - Mitochondrial antibodyAnti-Friedreich Ataxia Protein antibodyAnti-Fxn Cleaved Into - Frataxin Intermediate Form antibodyAnti-I-Fxn - Frataxin(56-210 antibodyAnti-M56-Fxn - Frataxin(78-210 antibodyAnti-D-Fxn antibodyAnti-M78-Fxn - Frataxin Mature Form antibodyAnti-Frataxin(81-210 antibodyAnti-M81-Fxn - Extramitochondrial Frataxin antibodyAnti-FXN antibodyAnti-FRDA antibodyAnti-X25 antibody |
Information sourced from Uniprot.org
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
