BackgroundThis nuclear gene encodes a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA from 8-33 repeats to >90 repeats results in Friedreich ataxia. Alternative splicing results in multiple transcript variants.
DescriptionFXN/Frataxin Polyclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
SpecificityHuman, Mouse
IsotypeIgG
Uniprot IDQ16595
PurificationAffinity Purified
ImmunogenRecombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN / Frataxin (NP_000135.2).
StorageShipped at 4°C. Store at -20°C. Avoid freeze / thaw cycles.
Alternative NamesFXN; CyaY; FA; FARR; FRDA; X25; frataxin
ApplicationWB; Recommended dilution: WB 1:500 - 1:2000