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Product Name
FXN antibody
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Description
FXN Rabbit Polyclonal antibody. Positive WB detected in MDA-MB-453s cells, human cerebellum tissue, human heart tissue, Jurkat cells, K-562 cells, Raji cells. Observed molecular weight by Western-blot: 14kd
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Tested applications
ELISA, WB
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Species reactivity
Human; other species not tested.
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Alternative names
CyaY antibody; d FXN antibody; FARR antibody; frataxin antibody; Frataxin(81 210) antibody; Frataxin antibody; mitOChondrial antibody; FRDA antibody; Friedreich ataxia protein antibody; FXN antibody; i FXN Frataxin(56 210) antibody; m56 FXN Frataxin(78 210) antibody; m78 FXN Frataxin mature form antibody; m81 FXN antibody; X25 antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of FXN recombinant protein (Accession Number: NM_000144). Purification method: Antigen affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:500-1:5000
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Validations
MDA-MB-453s cells were subjected to SDS PAGE followed by western blot with Catalog No:110751(FXN antibody) at dilution of 1:500
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Background
FXN, also named as FRDA, X25, m81-FXN, d-FXN, m78-FXN and i-FXN, belongs to the frataxin family. It promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. FXN may play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+; the oligomeric form but not the monomeric form has in vitro ferroxidase activity. FXN is cleaved to be 4 chains. The mature form of FXN is 14kd or 18kd.
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References
- Choquet K, Zurita-Rendón O, La Piana R. Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA. Brain : a journal of neurology. 139(Pt 3):e19. 2016.
- Nabhan JF, Wood KM, Rao VP. Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia. Scientific reports. 6:20019. 2016.
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