Frataxin Polyklonale Antilichaam

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.

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.

Human, Mouse, Rat

110-210/210

Frataxin

Unconjugated

KLH conjugated synthetic peptide derived from human Frataxin

WB, IHC-P, IHC-F, IF

Purified by Protein A.

1µg/µl

WB (WB=1:500-2000), IHC-P (IHC-P=1:100-500), IHC-F (IHC-F=1:100-500), IF (IF=1:100-500)

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Unmodified

Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.