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
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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