Anti-Frataxin抗体[17A11] (ab113691)
Key features and details
- Mouse monoclonal [17A11] to Frataxin
- Suitable for: WB, ICC/IF, Flow Cyt, IHC-P
- Reacts with: Mouse, Rat, Cow, Human, Recombinant fragment
- Isotype: IgG1
Related conjugates and formulations
概述
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产品名称
Anti-Frataxin抗体[17A11]
参阅全部 Frataxin 一抗 -
描述
小鼠单克隆抗体[17A11] to Frataxin -
宿主
Mouse -
经测试应用
适用于: WB, ICC/IF, Flow Cyt, IHC-Pmore details -
种属反应性
与反应: Mouse, Rat, Cow, Human, Recombinant fragment -
免疫原
Recombinant fragment within Human Frataxin aa 1-120. The exact sequence is proprietary.
Database link: Q16595 -
阳性对照
- WB: heart, liver tissue lysate. IHC-P: heart muscle, liver, kidney tissue (FFPE).
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常规说明
This antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact orders@abcam.com.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Product was previously marketed under the MitoSciences sub-brand.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C. -
存储溶液
pH: 7.5
Preservative: 0.02% Sodium azide
Constituent: HEPES buffered saline -
Concentration information loading...
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纯化说明
The antibody was produced in vitro using hybridomas grown in serum-free medium, and then purified by affinity purification. -
克隆
单克隆 -
克隆编号
17A11 -
同种型
IgG1 -
轻链类型
kappa -
研究领域
相关产品
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Alternative Versions
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Compatible Secondaries
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Conjugation kits
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Isotype control
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab113691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 | (2) |
Use a concentration of 4 µg/ml. Detects a band of approximately 14 kDa (predicted molecular weight: 14 kDa).
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ICC/IF | (1) |
Use a concentration of 1 µg/ml.
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Flow Cyt |
Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody. |
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IHC-P |
Use a concentration of 10 µg/ml.
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说明 |
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WB
Use a concentration of 4 µg/ml. Detects a band of approximately 14 kDa (predicted molecular weight: 14 kDa). |
ICC/IF
Use a concentration of 1 µg/ml. |
Flow Cyt
Use a concentration of 1 µg/ml. ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody. |
IHC-P
Use a concentration of 10 µg/ml. |
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: 505694 Cow
- Entrez Gene: 2395 Human
- Entrez Gene: 14297 Mouse
- Entrez Gene: 499335 Rat
- Omim: 606829 Human
- SwissProt: Q05B87 Cow
- SwissProt: Q16595 Human
- SwissProt: O35943 Mouse
see all -
别名
- CyaY antibody
- d-FXN antibody
- FA antibody
see all
图片
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All lanes : Anti-Frataxin antibody [17A11] (ab113691)
Lane 1 : Recombinant frataxin
at 0.1 µg
Lane 2 : Human heart mitochondria
at 15 µg
Lane 3 : Human liver mitochondria
at 15 µg
Lane 4 : HepG2 whole cell lysate
at 15 µg
Lane 5 : Bovine heart mitochondria
at 15 µg
Lane 6 : H9C2 rat whole cell lysate
at 15 µg
Lane 7 : MEF mouse embryonic fibroblast
at 15 µg
Lane 8 : Mouse liver mitochondria
at 15 µg
Lane 9 : Rat liver mitochondria
at 15 µg
Predicted band size: 14 kDa -
ab113691 stained fibroblast cells. The cells were paraformaldehyde fixed (4%, 20 min) and Triton X-100 permeabilized (0.1%, 15min). The cells were incubated with the antibody (ab113691, 1µg/ml) for 2h at room temperature or over night at 4°C. The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1h. 10% Goat serum was used as the blocking agent for all blocking steps. The target protein locates to the mitochondrial matrix.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [17A11] (ab113691)
IHC image of Frataxin staining in Human normal heart muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab113691, 10µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
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Normal human lymphoblast cells were stained with 1 µg/mL ab113691 (blue) or an equal amount of an isotype control antibody (red) and analyzed by flow cytometry.
数据表及文件
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SDS download
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Datasheet download
文献 (17)
ab113691 被引用在 17 文献中.
- Rojsajjakul T et al. Liquid Chromatography-Mass Spectrometry Analysis of Frataxin Proteoforms in Whole Blood as Biomarkers of the Genetic Disease Friedreich's Ataxia. Anal Chem 95:4251-4260 (2023). PubMed: 36800320
- Medina-Carbonero M et al. Mice harboring the FXN I151F pathological point mutation present decreased frataxin levels, a Friedreich ataxia-like phenotype, and mitochondrial alterations. Cell Mol Life Sci 79:74 (2022). PubMed: 35038030
- Rodden LN et al. DNA methylation in Friedreich ataxia silences expression of frataxin isoform E. Sci Rep 12:5031 (2022). PubMed: 35322126
- Wang Q et al. Simultaneous Quantification of Mitochondrial Mature Frataxin and Extra-Mitochondrial Frataxin Isoform E in Friedreich's Ataxia Blood. Front Neurosci 16:874768 (2022). PubMed: 35573317
- Terzi EM et al. Iron-sulfur cluster deficiency can be sensed by IRP2 and regulates iron homeostasis and sensitivity to ferroptosis independent of IRP1 and FBXL5. Sci Adv 7:N/A (2021). PubMed: 34039609