Frataxin Antibody / FXN

SKU: orb534290

Description

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]

Images & Validation

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Tested Applications	IHC-P, WB
Dilution range	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)
Reactivity	Human
Application 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.

Key Properties

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Antibody Type	Primary Antibody
Host	Mouse
Clonality	Monoclonal
Isotype	Mouse IgG2b, kappa
Clone No.	FDAX-1
Immunogen	Amino acids 57-210 from the human protein were used as the immunogen for this Frataxin antibody.
Conjugation	Unconjugated

Storage & Handling

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Storage	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.
Disclaimer	For research use only

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Western blot testing of Fascin antibody and human HepG2 cell lysate (clone FDAX-1). Expected molecular weight: ~23 kDa (full length), ~14 kDa (mature).

IHC testing of FFPE human pancreas with Frataxin antibody (clone FDAX-1). Required HIER: boil tissue sections in pH9 10mM Tris with 1mM EDTA for 10-20 min and allow to cool before testing.

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UniProt

Q16595

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Protocol Information

Western Blot (IB, immunoblot)

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IHC-P

Immunohistochemistry Paraffin

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