Overview

  • Product name
    Anti-ETFDH antibody [3H2BG1]
    See all ETFDH primary antibodies
  • Description
    Mouse monoclonal [3H2BG1] to ETFDH
  • Tested applications
    Suitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Cow, Human
  • Immunogen

    Human native ETFDH protein

  • Positive control
    • 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.
  • General notes

    This antibody clone is manufactured by Abcam.

    Product was previously marketed under the MitoSciences sub-brand.

Properties

Applications

Our Abpromise guarantee covers the use of ab126576 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 68 kDa.
IHC-P Use a concentration of 5 µg/ml.

Target

  • Function
    Accepts electrons from ETF and reduces ubiquinone.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the ETF-QO/fixC family.
    Contains 1 4Fe-4S ferredoxin-type domain.
  • Cellular localization
    Mitochondrion inner membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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

Images

  • 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
    GAM-HRP at 1/3000 dilution

    Predicted band size : 68 kDa
  • 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.

References

This product has been referenced in:
  • 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 . Read more (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 . Read more (PubMed: 23727839) »

See all 3 Publications for this product

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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