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>   首页   >   产品   >   一抗   >   代谢   >   FXN Antibody (C-term)   

FXN Antibody (C-term)

Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - FXN Antibody (C-term) AP6409B
    Western blot analysis of anti-FXN Antibody (C-term) (Cat#AP6409b) in 293 cell line lysates (35ug/lane). FXN (arrow) was detected using the purified Pab.
  • 1 - FXN Antibody (C-term) AP6409B
    Western blot analysis of FXN (arrow) using rabbit polyclonal FXN Antibody (C-term) (RB12571). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the FXN gene (Lane 2) (Origene Technologies).
  • 14 - FXN Antibody (C-term) AP6409B
    Formalin-fixed and paraffin-embedded human lung carcinoma tissue reacted with FXN antibody (C-term) (Cat.#AP6409b), 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.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, E
Primary Accession Q16595
Other Accession O35943
Reactivity Human
Predicted Mouse
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 23135 Da
Antigen Region 143-172 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 143-172 amino acids from the C-terminal region of human FXN.
Dilution WB~~1:1000
IHC-P~~1:100~500
E~~Use at an assay dependent concentration.
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 (C-term) 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:12785837, PubMed:24971490). 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

For Research Use Only. Not For Use In Diagnostic Procedures.

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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