Home >> Antibodies >> Frataxin (Mitochondrial Marker)

Frataxin (Mitochondrial Marker)

Recombinant Mouse Monoclonal Antibody [Clone rFXN/2124]

Printable

Key References

Bioinformatics

Catalog #

Size

20 ug

100 ug

Price (USD)

199.00

459.00

Formulation

Purified Ab with BSA and Azide at 200ug/ml

Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Performance Guarantee

Catalog No

Size

20 ug

100 ug

Formulation

Purified Ab with BSA and Azide at 200ug/ml

Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Price (USD)

199.00

459.00

Printable

Key References

Bioinformatics

Product Details

Synonyms

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

Positive Control

HepG2, HL-60 or HEK293 cells. Pancreas and Liver.

Known Applications & Suggested Dilutions

ELISA More Details

ELISA (Use Ab at 2-4ug/ml for coating) (Order Ab without BSA)
Optimal dilution for a specific application should be determined.

Immunogen

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

Cellular Localization

Cytoplasmic (Mitochondria)

Species Reactivity

Human.

Host / Ig Isotype

Mouse / IgG1, kappa

Mol. Weight of Antigen

14kDa

Specificity & Comments

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 suggesting 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.