|Tested species reactivity||Human|
|Published species reactivity||Not Applicable|
|Host / Isotype||Mouse / IgG1|
|Immunogen||Human frataxin (full length mature form, residues 56-210)|
|Storage buffer||HEPES buffered saline|
|Contains||0.02% sodium azide|
|Storage Conditions||4° C, do not freeze|
|Tested Applications||Dilution *|
|Immunocytochemistry (ICC)||0.5 ug/ml|
|Immunofluorescence (IF)||Assay Dependent|
|Western Blot (WB)||3-5 ug/ml|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
|Western Blot (WB)||See 1 publications below|
FXN is 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 results in Friedreich ataxia.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
|Not Applicable||Not Cited||
Long intronic GAA repeats causing Friedreich ataxia impede transcription elongation.
45-6300 was used in western blot to report that FXN mRNA synthesis, but not turnover rates, is affected by an expanded GAA repeat tract
|Punga T,Bühler M||EMBO molecular medicine (2:120)||2010|