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
CAS Number
9007-83-4
Specifications
Western blot: 1-2 µg/mL, Immunohistochemistry (FFPE) : 1-2 µg/mL for 30 min at RT, Prediluted IHC only format: incubate for 30 min at RT (1)
UniProt
Q16595
Host
Mouse
Reactivity
Human
Immunogen
Amino acids 57-210 from the human protein were used as the immunogen for this Frataxin antibody.
Clonality
Monoclonal
Isotype
IgG2b κ
Clone
FDAX-1
Applications
IHC-P, WB
Purity
Protein G affinity chromatography
Format
Purified
Buffer
0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide
Reconstitution
Store the Frataxin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Limitations
This Frataxin antibody is available for research use only.
Storage Conditions
Store the Frataxin antibody at 2-8°C (with azide) or aliquot and store at -20°C or colder (without azide) .
Formulation
0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced) and 0.05% sodium azide
Location
Cytoplasmic
Image Legend
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