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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
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Frataxin (FXN) is a mitochondrial protein involved in iron homeostasis, iron-sulfur cluster assembly, oxidative metabolism, and mitochondrial respiratory function. FXN Antibody / Mitochondrial Iron Metabolism Antibody recognizes a conserved mitochondrial matrix-associated protein that contributes to cellular energy regulation and protection against mitochondrial oxidative stress-associated damage.
FXN antibody, also referred to as Frataxin antibody and Friedreich ataxia protein antibody in the literature, is widely used in mitochondrial biology, neurodegeneration, metabolic signaling, and iron metabolism research applications. Monoclonal clone FXN/2124 supports investigation of mitochondrial iron handling pathways and iron-sulfur cluster-associated metabolic processes linked to neurologic and metabolic disease biology.
Frataxin is primarily localized within mitochondria where it interacts with iron-sulfur cluster assembly machinery including ISCU scaffold proteins, NFS1 cysteine desulfurase complexes, and mitochondrial iron transport-associated regulatory pathways. Through these interactions, FXN contributes to biogenesis of iron-sulfur-containing enzymes required for oxidative phosphorylation, electron transport chain activity, and mitochondrial metabolic homeostasis.
Reduced FXN expression is strongly associated with Friedreich ataxia, an inherited neurodegenerative disorder characterized by progressive neurologic dysfunction, cardiomyopathy, mitochondrial impairment, and iron accumulation within affected tissues. Impaired Frataxin activity disrupts mitochondrial iron regulation and oxidative metabolism, contributing to reactive oxygen species generation and defective energy production pathways.
In addition to neurologic disease research, FXN has become increasingly relevant in studies examining mitochondrial dysfunction, oxidative stress adaptation, metabolic regulation, and aging-associated cellular processes. Altered mitochondrial iron homeostasis and iron-sulfur cluster integrity influence multiple metabolic signaling pathways linked to cellular stress responses and mitochondrial quality control mechanisms.
Western blot analysis with FXN antibodies commonly identifies mature Frataxin near 14-18 kDa depending on precursor processing state and mitochondrial maturation-associated cleavage. Immunohistochemistry staining may demonstrate predominantly granular cytoplasmic staining patterns consistent with mitochondrial localization within metabolically active cellular populations. Because FXN is enriched in tissues with high mitochondrial demand, staining intensity may vary according to metabolic activity and tissue-specific energy requirements.
Analysis of HuProt(TM) protein microarray containing more than 19,000 full-length human proteins demonstrated selective recognition of FXN by clone FXN/2124, supporting strong target specificity relative to unrelated proteins present on the array. The observed microarray specificity profile further supports the utility of this monoclonal antibody for selective Frataxin detection in mitochondrial and metabolic research applications.
Together, the available immunohistochemistry, western blot, and HuProt microarray specificity data support the use of FXN antibody clone FXN/2124 for investigating mitochondrial iron metabolism, iron-sulfur cluster biogenesis, and Frataxin-associated metabolic signaling pathways.
Explore additional Metabolism Antibodies targeting mitochondrial regulatory proteins, iron-sulfur cluster pathways, and oxidative metabolism-associated signaling proteins.
Optimal dilution of the FXN Antibody / Mitochondrial Iron Metabolism Antibody should be determined by the researcher.
1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.
Amino acids 57-210 from the human protein were used as the immunogen for this FXN antibody.
Store the FXN antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
FXN antibody, Frataxin antibody, Friedreich ataxia protein antibody, mitochondrial iron metabolism protein antibody, iron-sulfur cluster protein antibody
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