货号 |
bs-9601R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-Frataxin antibody |
中文名称 |
Rabbit Anti-Frataxin antibody |
研究领域 |
肿瘤,心血管,细胞生物,免疫学,神经生物学,信号转导,线抗体, |
英文别名 |
X25; CyaY; d-FXN; FA antibody FARR; Frataxin mature form; Frataxin(81-210); FRDA; FRDA_HUMAN; Friedreich ataxia protein; FXN; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; MSF01; MSF31; MSF42. |
反应物种(已验证) |
Mouse,Rat |
反应物种(预测) |
Human,Chicken,Dog,Pig,Cow,Horse |
产品应用(可推荐) |
WB,IHC,ICC,IF,ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC=1:100-500, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
19 |
细胞定位 |
细胞浆 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human Frataxin |
抗原表位 |
110-210/210 |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. |
Tissue |
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. |
SIMILARITY |
Belongs to the frataxin family. |
SUBUNIT |
Belongs to the frataxin family. |
Function |
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 ox |
Post-translational |
Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additio |
DISEASE |
Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually m |
SWISS |
Q16595 |
Gene ID |
2395 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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. |