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Frataxin Antibody / FXN

Reduced expression of frataxin is the cause of Friedreich's ataxia (FRDA), a lethal neurodegenerative disease. The reduction in frataxin gene expression may be attributable from either the silencing of transcription of the frataxin gene because of epigenetic modifications in the chromosomal entity or from the inability of splicing the expanded GAA repeats in the first intron of the pre-mRNA as seen in bacteria and human cells or both. The expansion of intronic trinucleotide repeat GAA results in Friedreich's ataxia. [Wiki]

Product Specifications

UniProt

Q16595

Reactivity

Human

Immunogen

Amino acids 57-210 from the human protein were used as the immunogen for this Frataxin antibody.

Clonality

Monoclonal

Clone

FDAX-1

Conjugation

Unconjugated

Field of Research

Metabolism Research

Purification

Protein G affinity chromatography

Dilution

Western blot: 1-2ug/ml, Immunohistochemistry (FFPE) : 1-2ug/ml for 30 min at RT, Prediluted IHC only format: incubate for 30 min at RT (1)

Storage Conditions

Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Notes

For research use only.

Applications Notes

Optimal dilution of the Frataxin antibody should be determined by the researcher.1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.

Tested Applications

IHC-P, WB

Host or Source

Mouse

Preservative

1 mg/ml in 1X PBS; rAlbumin free, sodium azide free

Isotype

Mouse IgG2b, kappa

Frequently Asked Questions

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