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Anti-ETFDH抗体[3H2BG1] (ab126576)

  • Datasheet
  • SDS
Submit a review Submit a question References (4)

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Western blot - Anti-ETFDH antibody [3H2BG1] (ab126576)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ETFDH antibody [3H2BG1] (ab126576)

Key features and details

  • Mouse monoclonal [3H2BG1] to ETFDH
  • Suitable for: WB, IHC-P
  • Reacts with: Mouse, Rat, Cow, Human
  • Isotype: IgG1

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概述

  • 产品名称

    Anti-ETFDH抗体[3H2BG1]
    参阅全部 ETFDH 一抗
  • 描述

    小鼠单克隆抗体[3H2BG1] to ETFDH
  • 宿主

    Mouse
  • 经测试应用

    适用于: WB, IHC-Pmore details
  • 种属反应性

    与反应: Mouse, Rat, Cow, Human
  • 免疫原

    The details of the immunogen for this antibody are not available.

  • 阳性对照

    • Human cell, heart and liver lysates, Rat liver lysates, Mouse liver lysates, Bovine heart mitochondria lysate. This antibody gave a positive result in IHC in the following FFPE tissue: Human normal heart muscle.
  • 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

  • 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: 99% HEPES buffered saline
  • Concentration information loading...
  • 纯度

    Ammonium Sulphate Precipitation
  • 纯化说明

    Produced in vitro using hybridomas grown in serum-free medium, and then concentrated by ammonium sulfate precipitation. Near homogeneity as judged by SDS-PAGE.
  • 克隆

    单克隆
  • 克隆编号

    3H2BG1
  • 同种型

    IgG1
  • 轻链类型

    kappa
  • 研究领域

    • Tags & Cell Markers
    • Subcellular Markers
    • Organelles
    • Mitochondria
    • Signal Transduction
    • Metabolism
    • Mitochondrial
    • Metabolism
    • Pathways and Processes
    • Mitochondrial Metabolism
    • Mitochondrial markers
    • Metabolism
    • Types of disease
    • Cancer

相关产品

  • Compatible Secondaries

    • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
    • Goat Anti-Mouse IgG H&L (HRP) (ab205719)
  • Isotype control

    • Mouse IgG1, kappa monoclonal [15-6E10A7] - Isotype Control (ab170190)

应用

The Abpromise guarantee

Abpromise™承诺保证使用ab126576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
WB
Use a concentration of 1 µg/ml. Predicted molecular weight: 68 kDa.
IHC-P
Use a concentration of 5 µg/ml.
说明
WB
Use a concentration of 1 µg/ml. Predicted molecular weight: 68 kDa.
IHC-P
Use a concentration of 5 µg/ml.

靶标

  • 功能

    Accepts electrons from ETF and reduces ubiquinone.
  • 疾病相关

    Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C) [MIM:231680]. GA2C is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids.
  • 序列相似性

    Belongs to the ETF-QO/fixC family.
    Contains 1 4Fe-4S ferredoxin-type domain.
  • 细胞定位

    Mitochondrion inner membrane.
  • Target information above from: UniProt accession Q16134 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • 数据库链接

    • Entrez Gene: 768074 Cow
    • Entrez Gene: 2110 Human
    • Entrez Gene: 66841 Mouse
    • Entrez Gene: 295143 Rat
    • Omim: 231675 Human
    • SwissProt: Q2KIG0 Cow
    • SwissProt: Q16134 Human
    • SwissProt: Q921G7 Mouse
    • SwissProt: Q6UPE1 Rat
    • Unigene: 155729 Human
    • Unigene: 28336 Mouse
    • Unigene: 37277 Rat
    see all
  • 别名

    • Electron transfer flavoprotein ubiquinone oxidoreductase antibody
    • Electron transfer flavoprotein-ubiquinone oxidoreductase antibody
    • electron transferring flavoprotein dehydrogenase antibody
    • Electron-transferring-flavoprotein dehydrogenase antibody
    • ETF dehydrogenase antibody
    • ETF QO antibody
    • ETF ubiquinone oxidoreductase antibody
    • ETF-QO antibody
    • ETF-ubiquinone oxidoreductase antibody
    • ETFD_HUMAN antibody
    • Etfdh antibody
    • MADD antibody
    • mitochondrial antibody
    see all

图片

  • Western blot - Anti-ETFDH antibody [3H2BG1] (ab126576)
    Western blot - Anti-ETFDH antibody [3H2BG1] (ab126576)
    All lanes : Anti-ETFDH antibody [3H2BG1] (ab126576) at 1 µg/ml

    Lane 1 : Human heart lysate at 20 µg
    Lane 2 : Human liver lysate at 20 µg
    Lane 3 : Human cell lysate at 20 µg
    Lane 4 : Rat liver lysate at 20 µg
    Lane 5 : Mouse liver lysate at 20 µg
    Lane 6 : Bovine heart mitochondria
    at 10 µg

    Secondary
    All lanes : GAM-HRP at 1/3000 dilution

    Predicted band size: 68 kDa



    The high background signal in Mouse tissue sample was caused by the direct reaction between the Mouse IgG in Mouse tissue preps and the goat anti-Mouse secondary antibody.
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ETFDH antibody [3H2BG1] (ab126576)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ETFDH antibody [3H2BG1] (ab126576)

    IHC image of ETFDH 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 ab126576, 5µg/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.

实验方案

  • Mouse on Mouse staining protocol

Click here to view the general protocols

数据表及文件

  • SDS download

  • Datasheet download

    Download

文献 (4)

发表研究结果有使用 ab126576?请让我们知道,以便我们可以引用本数据表中的参考文章。

ab126576 被引用在 4 文献中.

  • Fisher-Wellman KH  et al. Respiratory Phenomics across Multiple Models of Protein Hyperacylation in Cardiac Mitochondria Reveals a Marginal Impact on Bioenergetics. Cell Rep 26:1557-1572.e8 (2019). PubMed: 30726738
  • Zhu M  et al. Riboflavin-responsive multiple Acyl-CoA dehydrogenation deficiency in 13 cases, and a literature review in mainland Chinese patients. J Hum Genet N/A:N/A (2014). Human . PubMed: 24522293
  • Cornelius N  et al. Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency. Hum Mol Genet 22:3819-27 (2013). WB ; Human . PubMed: 23727839
  • Orr AL  et al. A Refined Analysis of Superoxide Production by Mitochondrial sn-Glycerol 3-Phosphate Dehydrogenase. J Biol Chem 287:42921-35 (2012). WB . PubMed: 23124204

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