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.
This antibody clone is manufactured by Abcam.
Product was previously marketed under the MitoSciences sub-brand.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 68 kDa.
Use a concentration of 5 µg/ml.
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.
Belongs to the ETF-QO/fixC family. Contains 1 4Fe-4S ferredoxin-type domain.
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
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.
Zhu M et al. Riboflavin-responsive multiple Acyl-CoA dehydrogenation deficiency in 13 cases, and a literature review in mainland Chinese patients. J Hum GenetN/A:N/A (2014).
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 Genet22:3819-27 (2013).
Read more (PubMed: 23727839) »