Frataxin Polyclonal Antibody
Product Specifications
Background
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.The frataxin gene encodes a mitochondrial protein of the same name. Frataxin assembles into a stable homopolymer with iron-binding capabilities. When expressed in E. Coli human frataxin binds iron atoms at a rate of 10 iron atoms per 1 molecule of the frataxin polymer. Thus, frataxin appears to function in some capacity for iron-storage for the mitochondria. Frataxin may also function as an activator of oxidative phosphorylation to increase mitochondrial membrane potential and elevate cellular ATP. Frataxin is expressed in tissues with high metabolic activity including heart, liver and brown fat.
Synonyms
X25; CyaY; d-FXN; FA antibody FARR; Frataxin mature form; Frataxin81-210; FRDA; FRDA_HUMAN; Friedreich ataxia protein; FXN; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; MSF01; MSF31; MSF42.
Host
Rabbit
Cross Reactivity
Human, Mouse, Rat
Immunogen
110-210/210
Target
Frataxin
Clonality
Polyclonal
Isotype
IgG
Conjugation
Unconjugated
Source
KLH conjugated synthetic peptide derived from human Frataxin
Applications
WB, IHC-P, IHC-F, IF
Purification
Purified by Protein A.
Concentration
1µg/µl
Dilution
WB (WB=1:500-2000), IHC-P (IHC-P=1:100-500), IHC-F (IHC-F=1:100-500), IF (IF=1:100-500)
Buffer
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.
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