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

CAS Number

9007-83-4

Specifications

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

UniProt

Q16595

Host

Mouse

Reactivity

Human

Immunogen

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

Clonality

Monoclonal

Isotype

IgG2b κ

Clone

FDAX-1

Applications

IHC-P, WB

Purity

Protein G affinity chromatography

Format

Purified

Buffer

0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide

Reconstitution

Store the Frataxin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).

Limitations

This Frataxin antibody is available for research use only.

Storage Conditions

Store the Frataxin antibody at 2-8°C (with azide) or aliquot and store at -20°C or colder (without azide) .

Formulation

0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced) and 0.05% sodium azide

Location

Cytoplasmic

Image Legend

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