|Positive WB detected in||MDA-MB-453s cells, K-562 cells|
|Western Blot (WB)||WB : 1:500-1:2000|
|Sample-dependent, check data in validation data gallery|
14147-1-AP targets FXN in WB, IHC, ELISA applications and shows reactivity with human, mouse, rat samples.
|Tested Reactivity||human, mouse, rat|
|Cited Reactivity||human, mouse, rat|
|Host / Isotype||Rabbit / IgG|
|Immunogen||FXN fusion protein Ag5316|
|Calculated molecular weight||23 kDa|
|Observed molecular weight||14 kDa|
|GenBank accession number||BC048097|
|Gene ID (NCBI)||2395|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.|
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. FXN is expressed in the cytoplasm as a 210 amino acid (AA) precursor protein (pFXN; 23 KDa) that is translocated into mitochondria where it is processed by two consecutive steps into iFXN (FXN 42-210; 19 KDa) and finally mFXN (81-210; 14.2 KDa), which is functional. (PMID: 26704351, PMID: 26671574)
Biochim Biophys Acta
Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis.
Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich's Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System.
Dis Model Mech
Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia.
Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis.
Salidroside inhibits doxorubicin-induced cardiomyopathy by modulating a ferroptosis-dependent pathway.
Hum Mol Genet
Premature transcription termination at the expanded GAA repeats and aberrant alternative polyadenylation contributes to the Frataxin transcriptional deficit in Friedreich's ataxia.
The reviews below have been submitted by verified Proteintech customers who received an incentive forproviding their feedback.
Mariana (Verified Customer) (01-06-2021)
Failed to detect endogenous FXN in IPSC derived Cardiomyocytes from Friedreich Ataxia patients and controls.
Zhuqing (Verified Customer) (11-26-2020)
In mouse cells, it can detect mature frataxin.
Daniel (Verified Customer) (08-20-2019)
Works fine for WB in mouse brain and heart tissues.