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FXN (基因名), Frataxin, mitochondrial (蛋白名), frda_human.

产品名称:

FXN Antibody

货号:

P15247Rb-h

商标:

EIAab^®

监管等级:

RUO

别名:

Friedreich ataxia protein, Fxn, FRDA, X25

序列号:

Q16595

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:

Polyclonal

亚型:

Rabbit IgG

应用:

推荐产品:

Goat Anti-Rabbit IgG(H+L) HRP-conjugated Antibody

研究领域:

规格 & 价格:

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Human FXN Polyclonal Antibody

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产品说明书

数据表:

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R&D 技术数据

IHC-P analysis of human liver cancer using FXN antibody at a dilution of 1:200.

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.

数据库链接

UniGene:

Hs.20685

SMR:

Q16595

STRING:

9606.ENSP00000366482

KEGG:

hsa:2395

MIM: