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)
Formulation
1 mg/ml in 1X PBS; BSA free, sodium azide free
Host
Mouse
Immunogen Region
Amino acids 57-210 from the human protein were used as the immunogen for this Frataxin antibody.
Isotype
Mouse IgG2b, kappa
Species Reactivity
Human
Note
Optimal dilution of the Frataxin 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.
Uniprot
Q16595
Format
Purified
Purity
Protein G affinity chromatography
Storage
Store the Frataxin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Applications
WB, IHC-P
Description
Weste 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)