免疫原
Synthesized peptide derived from human FXN
特异性
This antibody detects endogenous levels of FXN at Human, Mouse,Rat
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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.
基因名称（Gene Name）
FXN FRDA X25
蛋白名称
Frataxin;mitochondrial (Friedreich ataxia protein) (Fxn) [Cleaved into: Frataxin intermediate form (i-FXN);Frataxin(56-210) (m56-FXN);Frataxin(78-210) (d-FXN) (m78-FXN);Frataxin mature form (Frataxin(81-210)) (m81-FXN)]
其他名称
CyaY;d-FXN;FA;FARR;Frataxin mature form;Frataxin(81-21);FRDA;FRDA_HUMAN;Friedreich ataxia protein;Fxn;i-FXN;m56-FXN;m78-FXN;m81-FXN;MGC57199;X25;FXN FRDA X25;Frataxin;mitochondrial (Friedreich ataxia protein) (Fxn) [Cleaved into: Frataxin intermediate form (i-FXN);Frataxin(56-210) (m56-FXN);Frataxin(78-210) (d-FXN) (m78-FXN);Frataxin mature form (Frataxin(81-210)) (m81-FXN)]
细胞定位
Mitochondrion . Cytoplasm, cytosol . PubMed:18725397 reports localization exclusively in mitochondria. .
组织表达
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
储存（Storage）
-15°C to -25°C/1 year(Do not lower than -25°C)
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