Description: Frataxin is a nuclear-encoded mitochondrial protein implicated in Friedreich's ataxia (FRDA), a human autosomal recessive neurodegenerative disease. Lack of Frataxin causes iron to accumulate in the mitochondrial matrix suggesting that Frataxin is involved in mitochondrial iron homeostasis and possibly in iron transport. Frataxin has an alpha-beta fold consisting of two helices flanking an antiparallel beta sheet. Primary antibodies are available purified, or with a selection of fluorescent CF® Dyes and other labels. CF® Dyes offer exceptional brightness and photostability. Note: Conjugates of blue fluorescent dyes like CF®405S and CF®405M are not recommended for detecting low abundance targets, because blue dyes have lower fluorescence and can give higher non-specific background than other dye colors.
Conjugate: Purified, with BSA
Concentration: 0.2 mg/mL
Storage buffer: PBS, 0.05% BSA, 0.05% azide
Product Origin: Animal
Clone: rFXN/2124
Entrez Gene ID: 2395
Immunogen: Recombinant human Frataxin protein fragment (around aa 57-210) (exact sequence is proprietary)