epitope corresponding to amino acids 56-210 mapping at the C-terminus of Frataxin of human origin
Modification Notes
Friedreich ataxia is a progressive neurodegenerative disorder caused by loss of function mutations in the frataxin gene. The human frataxin gene maps to chromosome 9q13 (1).The frataxin gene encodes a mitochondrial protein of the same name (2). Frataxin assembles into a stable homopolymer with iron-binding capabilities (3). When expressed in E. Coli, human frataxin binds iron atoms at a rate of 10 iron atoms per 1 molecule of the frataxin polymer (3). Thus, frataxin appears to function in some capacity for iron-storage for the mitochondria (3). Frataxin may also function as an activator of oxidative phosphorylation to increase mitochondrial membrane potential and elevate cellular ATP (4). Frataxin is expressed in tissues with high metabolic activity including heart, liver and brown fat (5).
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFELISA
recommended for detection of Frataxin of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including porcine:
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Alternative Names
epitope corresponding to amino acids 56-210 mapping at the C-terminus of Frataxin of human origin
Tissue Specificity
epitope corresponding to amino acids 56-210 mapping at the C-terminus of Frataxin of human origin