Frataxin is a small protein, localized to the mitochondrion.
The function of frataxin is not entirely clear, but it seems to be involved in assembly of iron-sulfur clusters.
It has been proposed to act as either an iron Chaperone (protein) or an iron storage protein.Reduced expression of frataxin is the cause of Friedrich's ataxia (FRDA), a lethal neurodegenerative disease.
This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family.
The protein functions in regulating mitochondrial iron transport and respiration.
The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia.
Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:100-1:5000Western Blot: 1:50-400Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100Immunocytochemistry: 1:50-500Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes human FXN.
Isotype：IgG
Calc Applications Abbrev：E IC IHC WB
Calc Crossreactivity：Hu
Immunogen：Recombinant protein corresponding to Ser57-Leu198 of human FXN, expressed in E. coli.
