重组Anti-Frataxin抗体[EPR6107] (ab124680)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR6107] to Frataxin
- Suitable for: WB
- Reacts with: Human
Related conjugates and formulations
概述
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产品名称
Anti-Frataxin抗体[EPR6107]
参阅全部 Frataxin 一抗 -
描述
兔单克隆抗体[EPR6107] to Frataxin -
宿主
Rabbit -
特异性
We suggest optimizing experimental protocols (increasing lysate amount, using lower dilution or higher sensitivity ECL substrate) to improve results.
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经测试应用
适用于: WBmore details
不适用于: IHC-P -
种属反应性
与反应: Human -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- WB: Human fetal heart tissue, human skin, His tagged Human FXN recombinant protein (aa 82-210), Human liver and hippocampus tissues, HCT116, Huh7 and SH-SY5Y whole cell lysates.
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常规说明
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Stable for 12 months at -20°C. -
解离常数(KD)
KD = 3.19 x 10 -10 M Learn more about KD -
存储溶液
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 0.05% BSA, 59% PBS -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR6107 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Isotype control
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab124680于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB |
1/1000 - 1/10000. Detects a band of approximately 17, 30 kDa (predicted molecular weight: 19, 23 kDa).
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说明 |
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WB
1/1000 - 1/10000. Detects a band of approximately 17, 30 kDa (predicted molecular weight: 19, 23 kDa). |
靶标
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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. -
组织特异性
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. -
疾病相关
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 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, FRDA 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. -
序列相似性
Belongs to the frataxin family. -
翻译后修饰
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 additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure. -
细胞定位
Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. - Information by UniProt
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数据库链接
- Entrez Gene: 2395 Human
- Omim: 606829 Human
- SwissProt: Q16595 Human
- Unigene: 20685 Human
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别名
- CyaY antibody
- d-FXN antibody
- FA antibody
see all
图片
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All lanes : Anti-Frataxin antibody [EPR6107] (ab124680) at 1/1000 dilution
Lane 1 : Human liver tissue lysate
Lane 2 : Human hippocampus tissue
Lane 3 : HCT116 (human colorectal carcinoma epithelial cell) whole cell lysate
Lane 4 : Huh7 (human hepatocellular carcinoma epithelial cell) whole cell lysate
Lane 5 : SH-SY5Y (human neuroblastoma epithelial cell) whole cell lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 19, 23 kDa
Observed band size: 14,17 kDa why is the actual band size different from the predicted?
Exposure time: 60 secondsBlocking buffer and concentration: 5% NFDM/TBST.
Diluting buffer and concentration: 5% NFDM/TBST.
This blot was developed using a high sensitivity ECL substrate.
For different forms of frataxin, you can refer to PMID: 17468497, PMID: 31279523, PMID: 17468497 etc.
ab181602 was used as a loading control.
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Anti-Frataxin antibody [EPR6107] (ab124680) at 1/1000 dilution + His tagged Human FXN recombinant protein (aa 82-210), 10ng
Secondary
Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 19, 23 kDa
Observed band size: 16 kDa why is the actual band size different from the predicted?
Exposure time: 60 secondsBlocking and diluting buffer and concentration: 5% NFDM/TBST.
ab213204 was used as a loading control.
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All lanes : Anti-Frataxin antibody [EPR6107] (ab124680) at 1/5000 dilution (purified)
Lane 1 : Human fetal heart tissue lysate
Lane 2 : Human skin lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/2000 dilution
Predicted band size: 19, 23 kDa
Observed band size: 30 kDa why is the actual band size different from the predicted?
Blocking and diluting buffer 5% NFDM/TBST
数据表及文件
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SDS download
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Datasheet download
文献 (1)
ab124680 被引用在 1 文献中.
- Weng L et al. Evaluation of antibodies for western blot analysis of frataxin protein isoforms. J Immunol Methods 474:112629 (2019). PubMed: 31279523