ANTI-FXN / frataxine produit en

Non disponible en dehors du Royaume-Uni et de l'Irlande

General description

This 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.

Immunogen

Recombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN / Frataxin (NP_000135.2).

Physical form

PBS with 0.02% sodium azide,50% glycerol,pH7.3.

Storage and Stability

Store at -20℃. Avoid freeze / thaw cycles.