- Tel: 858.663.9055
-
Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
Frataxin Antibody / Friedreich Ataxia Protein Antibody [clone FDAX-1] (V3997)
Email: info@nsjbio.com
| Catalog No | Formulation | Size | Price (USD) | ||
|---|---|---|---|---|---|
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V3997-100UG | 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide | 100 ug | 559 | |
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V3997-20UG | 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide | 20 ug | 259 | |
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V3997SAF-100UG | 1 mg/ml in 1X PBS; BSA free, sodium azide free | 100 ug | 559 | |
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V3997IHC-7ML | Prediluted in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide; *For IHC use only* | 7 ml | 559 |
| Availability | 1-3 business days |
| Species Reactivity | Human |
| Format | Purified |
| Host | Mouse |
| Clonality | Monoclonal (mouse origin) |
| Isotype | Mouse IgG2b, kappa |
| Clone Name | FDAX-1 |
| Purity | Protein G affinity chromatography |
| UniProt | Q16595 |
| Localization | Cytoplasmic |
| Applications | Western Blot : 1-2ug/ml Immunohistochemistry (FFPE) : 1-2ug/ml for 30 min at RT Prediluted IHC Only Format : incubate for 30 min at RT (1) |
| Limitations | This Frataxin Antibody / Friedreich Ataxia Protein Antibody is available for research use only. |
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Frataxin (FXN) is a mitochondrial matrix-associated protein involved in iron homeostasis, iron-sulfur cluster biogenesis, oxidative phosphorylation, and mitochondrial metabolic regulation. Frataxin Antibody / Friedreich Ataxia Protein Antibody recognizes a highly conserved mitochondrial protein that contributes to cellular energy production pathways and protection against mitochondrial oxidative stress-associated damage.
Frataxin antibody, also referred to as FXN antibody and Friedreich ataxia protein antibody in the literature, is widely used in mitochondrial biology, neurodegeneration, iron metabolism, and oxidative stress research applications. Monoclonal clone FDAX-1 supports investigation of mitochondrial iron handling pathways and Frataxin-associated metabolic signaling mechanisms linked to neurologic and mitochondrial disease biology.
FXN is synthesized as a precursor protein that undergoes mitochondrial import and proteolytic maturation to generate the active mature Frataxin protein localized within the mitochondrial matrix. Within mitochondria, Frataxin interacts with iron-sulfur cluster assembly machinery including ISCU scaffold proteins and cysteine desulfurase-associated complexes involved in generation of iron-sulfur cofactors required for oxidative metabolism and respiratory chain enzyme activity.
Reduced Frataxin expression is strongly associated with Friedreich ataxia, an inherited neurodegenerative disorder characterized by progressive ataxia, cardiomyopathy, mitochondrial dysfunction, and abnormal iron accumulation within affected tissues. Defective FXN-associated signaling contributes to impaired iron-sulfur cluster formation, oxidative stress accumulation, mitochondrial injury, and altered energy metabolism.
Because Frataxin functions at the intersection of mitochondrial metabolism and iron regulation, FXN has become increasingly important in studies examining oxidative stress adaptation, mitochondrial quality control pathways, neurodegenerative signaling, and metabolic homeostasis. Altered mitochondrial iron handling may additionally influence aging-associated pathways and cellular stress response signaling networks.
Western blot analysis with Frataxin antibodies commonly identifies mature FXN near approximately 14-15 kDa, while precursor forms may migrate at higher molecular weights depending on processing state and mitochondrial import-associated cleavage. Immunohistochemistry and immunofluorescence staining frequently demonstrate granular cytoplasmic patterns consistent with mitochondrial localization in metabolically active cellular populations.
Clone FDAX-1 supports investigation of Frataxin-associated mitochondrial pathways and contributes to studies examining iron-sulfur cluster assembly, oxidative phosphorylation regulation, and mitochondrial dysfunction-associated disease mechanisms. The established role of FXN in Friedreich ataxia and mitochondrial metabolism further supports the value of Frataxin antibodies in neurologic and metabolic research applications.
Together, the available biologic characterization supports the use of Frataxin antibody clone FDAX-1 for investigating mitochondrial iron homeostasis, oxidative metabolism-associated pathways, and Frataxin-linked mitochondrial signaling biology.
For additional Frataxin and mitochondrial metabolism research antibodies targeting iron-sulfur cluster assembly, oxidative phosphorylation pathways, and mitochondrial iron homeostasis proteins, explore the broader FXN Antibody page featuring monoclonal clone FXN/2124.
Optimal dilution of the Frataxin Antibody / Friedreich Ataxia Protein 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 Frataxin antibody.
Store the Frataxin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Frataxin antibody, FXN antibody, Friedreich ataxia protein antibody, mitochondrial iron homeostasis protein antibody, iron-sulfur cluster assembly protein antibody
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