catalog number :
MBS143636
products type :
Recombinant Protein
products full name :
Recombinant Human Frataxin
products short name :
Frataxin
products name syn :
Frataxin Human; Frataxin Human Recombinant; FXN; Friedreich ataxia protein; Frataxin mitochondrial; FRDA; X25; FA; CyaY; FARR; MGC57199; Frataxin; Frataxin
other names :
frataxin, mitochondrial isoform 1 preproprotein; Frataxin, mitochondrial; frataxin, mitochondrial; Friedreich ataxia protein; frataxin; Friedreich ataxia protein; Fxn
other gene names :
FXN; FXN; FA; X25; CyaY; FARR; FRDA; FRDA; X25; Fxn; i-FXN
uniprot entry name :
FRDA_HUMAN
purity :
Greater than 95.0% as determined by SDS-PAGE.
form :
The Frataxin solution contains 20mM Tris-HCl buffer (pH 8.0), 1mM DTT and 10% glycerol. Sterile Filtered colorless solution.
products categories :
RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins
products description :
Description: Frataxin Human Recombinant produced in E Coli is a single, non-glycosylated, polypeptide chain containing 190 amino acids (42-210 a.a.) and having a molecular mass of 21.1 kDa. The Frataxin is fused to a 20 amino acid His Tag at N-terminus and purified by proprietary chromatographic techniques. Introduction: Frataxin is a mitochondrial iron binding protein that is part of the FRATAXIN family. Frataxin functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Frataxin plays a role in iron homeostasis. Frataxin is an anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production.
ncbi acc num :
NP_000135.2
ncbi gb acc num :
NM_000144.4
ncbi mol weight :
21,416 Da
ncbi pathways :
HIF-2-alpha Transcription Factor Network Pathway 137956!!Metabolism Pathway 477135!!Metabolism Of Proteins Pathway 106230!!Mitochondrial Iron-sulfur Cluster Biogenesis Pathway 730343!!Mitochondrial Protein Import Pathway 576261
ncbi summary :
This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2009]