- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 R, H, M WB Rb Purified Polyclonal Antibody 描述 产品目录编号 AB15080 描述 Anti-Frataxin Antibody 背景信息 Frataxin is a nuclear encoded protein that is found in the mitochondria. This protein is not well understood but may be involved with iron sulfur clusters. In humans the gene is localized on chromosome 9 and is highly conserved during evolution. The gene is expressed in every cell, although in varying levels in different tissues and during development. The specific function of Frataxin is still unknown, but it has been shown to play a role in iron metabolism. Studies have demonstrated that the deletion of the Frataxin gene in yeast results in iron accumulation in mitochondria and loss of respiration. Recombinant human Frataxin has been shown to bind iron in vitro, and increased mitochondrial iron levels have been observed in patients with Friedreich's Ataxia (FRDA). 产品信息 格式 Purified 控制 - Human heart lysate.
演示 Rabbit polyclonal serum in buffer containing 0.05% NaN3. 应用 应用 This Anti-Frataxin Antibody is validated for use in WB for the detection of Frataxin. 主要应用 - Western Blotting
生物信息 免疫原品种 GST-tagged Frataxin corresponding to mature chain. 表位 mature chain 宿主 Rabbit 特异性 Cat. # AB15080 recognizes Frataxin. 品种反应性 RatHumanMouse Species Reactivity Note Human, Mouse and Rat. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_000135
Entrez基因汇总 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 occurs at this locus and two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq] 纯化方法 GST-depleted sera. UniProt编号 - Q16595
UniProt汇总 Function: Probably involved in iron homeostasis. Anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production. Ref.13
Subunit structure: Monomer.
Subcellular location: Mitochondrion. Note= Mitochondrial and extramitochondrial. Ref.13 Ref.8 Ref.9
Tissue specificity: Frataxin(81-210) is expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. Ref.4
Post-translational modification: Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein. Two forms exist, frataxin(56-210) and frataxin(81-210) which is the main form of mature frataxin.
Involvement in disease: Defects in FXN are the cause of Friedreich ataxia (FA) [MIM:229300]. FA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. Ref.6 Ref.7 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21
Sequence similarities: Belongs to the frataxin family.
产品使用声明 质量保证 Routinely evaluated by Western Blot on Human heart lysates.
Western Blot Analysis: 1:1,000 - 1:10,000 dilution of this lot detected Frataxin on 10 ug of human heart lysate
使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at -20ºC from date of receipt. 包装信息 数量 100 µL
上一件merck millipore产品：merck millipore,默克密理博,220320,Chetomin, Chaetomium sp. - CAS 1403-36-7 - Calbiochem
下一件merck millipore产品：merck millipore,默克密理博,04-262,Anti-Ubiquitinylated proteins Antibody, clone FK1
相关产品
merck millipore,默克密理博,MABD171,Anti-BMR2 Antibody, clone 3F6
merck millipore,默克密理博,ABN61,Anti-Alpha-dystrobrevin Antibody
merck millipore,默克密理博,569385,SQI-Pr - CAS 1022595-16-9 - Calbiochem
merck millipore,默克密理博,FCMAB152F,Milli-Mark Anti-FXR1 -FITC Antibody, clone 6BG10
merck millipore,默克密理博,220320,Chetomin, Chaetomium sp. - CAS 1403-36-7 - Calbiochem
merck millipore,默克密理博,04-262,Anti-Ubiquitinylated proteins Antibody, clone FK1
merck millipore,默克密理博,ABF215,Anti-UNC93B1 Antibody
merck millipore,默克密理博,CBL243,Anti-Glucose Transporter GLUT-4 Antibody
merck millipore,默克密理博,427275,Lactoferrin ELISA Kit
merck millipore,默克密理博,AB5338P,Anti-Cocaine and Amphetamine Related Transcript Antibody