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FXN Antibody (Center)

Purified Rabbit Polyclonal Antibody (Pab)

     
  • IF - FXN Antibody (Center) AP6409C
    Confocal immunofluorescent analysis of FXN Antibody (Center)(Cat#AP6409c) with WiDr cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).
    detail
  • WB - FXN Antibody (Center) AP6409C
    Western blot analysis of FXN Antibody (Center) (Cat.#AP6409c) in CEM cell line lysates (35ug/lane). FXN (arrow) was detected using the purified Pab.
    detail
  • IHC-P - FXN Antibody (Center) AP6409C
    Formalin-fixed and paraffin-embedded human colon carcinoma tissue reacted with FXN antibody (Center)(Cat.#AP6409c), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.
    detail
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
IF, WB, IHC-P, E
Primary Accession Q16595
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 23135 Da
Antigen Region 72-102 aa
Additional Information
Gene ID 2395
Other Names Frataxin, mitochondrial, Friedreich ataxia protein, Fxn, 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, FXN, FRDA, X25
Target/Specificity This FXN antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 72-102 amino acids from the Central region of human FXN.
Dilution IF~~1:10~50
WB~~1:1000
IHC-P~~1:10~50
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsFXN Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name FXN (HGNC:3951)
Synonyms FRDA, X25
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 (PubMed:24971490, PubMed:12785837). 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 (PubMed:24971490). Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly (PubMed:29576242). 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 (By similarity). 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 (PubMed:15641778). 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 (PubMed:11823441, PubMed:12755598). May function as an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation (PubMed:15247478). 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 (PubMed:15123683, PubMed:16239244).
Cellular Location [Frataxin mature form]: Mitochondrion
Tissue Location Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Research Areas
Citations (0)
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Background

FXN is 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 results in Friedreich ataxia.

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$ 365.00
$ 140.00
Cat# AP6409C
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