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Anti-Frataxin (Mitochondrial Marker) Monoclonal Antibody (Clone: rFXN/2124)

Frataxin is a nuclear-encoded mitochondrial protein implicated in Friedreich's ataxia (FRDA), a human autosomal recessive neurodegenerative disease. Lack of Frataxin causes iron to accumulate in the mitochondrial matrix sμggesting that Frataxin is involved in mitochondrial iron homeostasis and possibly in iron transport. Frataxin has an alpha-beta fold consisting of two helices flanking an antiparallel beta sheet.

Product Specifications

Product Name Alternative

CyaY; d-FXN; FARR; Frataxin mature form; Frataxin (81-210) ; FRDA; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; X25

Gene Name

FXN

Gene ID

2395

UniProt

Q16595

Host

Mouse

Reactivity

Human

Immunogen

Recombinant human Frataxin protein fragment (around aa 57-210) (exact sequence is proprietary)

Clonality

Monoclonal

Clone

RFXN/2124

Applications

ELISA

Components

200 μg/mL of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/mL.

Storage Conditions

Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous.

Applications Notes

ELISA (Use Ab at 2-4ug/ml for coating) (Order Ab without BSA)

Isotype

Mouse IgG1, kappa

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