FXN (基因名), Frataxin, mitochondrial (蛋白名), frda_human.
产品名称:
FXN Antibody
Frataxin,线粒体
货号:
M15247Rb-h
商标:
EIAab®
监管等级:
别名:
Friedreich ataxia protein, Fxn, FRDA, X25
反应性:
human
免疫原:
Recombinant human FXN protein ( R15247h )
形态:
Liquid
存储说明:
4℃ for frequent use, -20℃ to -70℃ for 6 months
缓冲液:
0.1M×PBS,50% glycerol,pH7.5
浓度:
200ug/ml
纯化方式:
Immunogen affinity purified
克隆性:
Monoclonal
亚型:
Mouse IgG
应用:
推荐产品:
研究领域:
-
R&D 技术数据
更多信息,请参阅手册,或联系我们的技术支持: tech@eiaab.com.
通用注释
亚单元:
Monomer (probable predominant form). Oligomer. Monomers and polymeric aggregates of >1 MDa have been isolated from mitochondria. A small fraction of heterologous overexpressed recombinant frataxin forms high-molecular wight aggregates that incoroprate iron. Interacts with LYRM4 AND HSPA9. Interacts with ACO1. Interacts with ISCU isoform 1 and isoform 2. Interacts with FECH; one iron-bound FXN monomer seems to interact with a FECH homodimer. Interacts with SDHA and SDHB. Interacts with ACO2; the interaction is dependent on citrate.
功能:
Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.
亚细胞位置:
Mitochondrion
Cytoplasm
Cytosol
PubMed:18725397 reports localization exclusively in mitochondria.
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