Frataxin Antibody / FXN
Reduced expression of frataxin is the cause of Friedreich's ataxia (FRDA), a lethal neurodegenerative disease. The reduction in frataxin gene expression may be attributable from either the silencing of transcription of the frataxin gene because of epigenetic modifications in the chromosomal entity or from the inability of splicing the expanded GAA repeats in the first intron of the pre-mRNA as seen in bacteria and human cells or both. The expansion of intronic trinucleotide repeat GAA results in Friedreich's ataxia. [Wiki]
Product Specifications
UniProt
Q16595
Reactivity
Human
Immunogen
Amino acids 57-210 from the human protein were used as the immunogen for this Frataxin antibody.
Clonality
Monoclonal
Clone
FDAX-1
Conjugation
Unconjugated
Field of Research
Metabolism Research
Purification
Protein G affinity chromatography
Dilution
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)
Storage Conditions
Maintain 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.
Notes
For research use only.
Applications Notes
Tested Applications
IHC-P, WB
Host or Source
Mouse
Preservative
1 mg/ml in 1X PBS; rAlbumin free, sodium azide free
Isotype
Mouse IgG2b, kappa
Frequently Asked Questions
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