Human Frataxin antibody

Frataxin, also known as FXN, is an iron binding protein which belongs to FRATAXIN family. It is an anti-apoptotic protein which prevents mitochondrial damage, reactive oxygen species production and act as either an iron chaperone or an iron storage protein. Frataxin is localized to the mitochondrion and regulating mitochondrial iron transport and respiration. Defects in this protein cause Friedreich ataxia.

Product Specifications

Product Name Alternative

CyaY, FA, FARR, FRDA, X25, FXN, Friedreich ataxia protein.

Host

Mouse

Antigen Species

Human

Reactivity

Human

Immunogen

Recombinant human Frataxin (42-210aa) purified from E. coli

Clonality

Monoclonal

Isotype

IgG1 κ

Clone

AT10E11

Conjugation

Unconjugated

Applications

ELISA, WB, ICC/IF

Purification Method

By protein-A affinity chromatography

Concentration

1 mg/mL (determined by BCA assay)

Additionnal Information

Frataxin, CyaY, FA, FARR, FRDA, X25, FXN, Friedreich ataxia protein., ATGA0440-10 µg, ATGA0440-20 µg, ATGA0440-50 µg, ATGA0440-100 µg, ATGA0440-250 µg, ATGA0440-500 µg, ATGA0440-1 mg, ATGA0440-10, ATGA0440-20, ATGA0440-50, ATGA0440-100, ATGA0440-250, ATGA0440-500, ATGA0440-1

References & Citations

Campuzano V., et al. (1996) Science. 271 (5254) : 1423-7. ; ; Shan Y., et al. (2007) Hum Mol Genet. 16 (8) : 929-41.

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol

Applications Notes

The antibody has been tested by ELISA, Western blot analysis, and ICC/IF to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Scientific Category

Signal Transduction

NCBI Accession Number

NP_000135

Uniprot Accession Number

Q16595

WB Description

The cell lysates (10 µg) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human Frataxin antibody (1:1000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system. ; ; Lane 1. : 293T cell lysate ; ; Lane 2. : Frataxin transfected 293T cell lysate

IF Description

ICC/IF analysis of Frataxin in A549 cell line, stained with DAPI (Blue) for nucleus staining and monoclonal anti-human Frataxin antibody (1:100) with goat anti-mouse IgG-Alexa fluor 488 conjugate (Green) .