Human Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial ELISA Kit | ETFDH elisa kit

Human Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial ELISA Kit

Cat. Num. AAA763588

• Gene Names: ETFDH; MADD; ETFQO
• Reactivity: Human
• Synonyms: Electron transfer flavoprotein-ubiquinone oxidoreductase; mitochondrial; Human Electron transfer flavoprotein-ubiquinone oxidoreductase; mitochondrial ELISA Kit; ETFDH (Electron transfer flavoprotein-ubiquinone oxidoreductase; mitochondrial)/ETF-QO/ETF-ubiquinone oxidoreductase/ETF dehydrogenase; ETFDH elisa kit

• Reactivity: Human
• Sequence Length: 617

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 78-5000pg/ml
• Sensitivity: 46.9pg/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data

Typical Standard Curve

Related Product Information for ETFDH elisa kit

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human ETFDH antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human ETFDH antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRPStreptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Human ETFDH amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Human ETFDHcan be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 119703746
• NCBI GeneID: 2110
• NCBI Accession #: NP_004444.2
• NCBI GenBank Nucleotide #: NM_004453.3
• Molecular Weight: 62,825 Da
• NCBI Official Full Name: electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial isoform 1
• NCBI Official Synonym Full Names: electron transfer flavoprotein dehydrogenase
• NCBI Official Symbol: ETFDH
• NCBI Official Synonym Symbols: MADD; ETFQO
• NCBI Protein Information: electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
• UniProt Protein Name: Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
• UniProt Gene Name: ETFDH
• UniProt Synonym Gene Names: ETF-QO; ETF-ubiquinone oxidoreductase; ETF dehydrogenase
• UniProt Entry Name: ETFD_HUMAN

NCBI Description

This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed. [provided by RefSeq, Aug 2013]

Uniprot Description

ETFDH: Accepts electrons from ETF and reduces ubiquinone. Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C). 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.

Protein type: Membrane protein, integral; EC 1.5.5.1; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 4q32-q35

Cellular Component: integral to mitochondrial inner membrane; mitochondrial matrix; mitochondrial membrane

Molecular Function: 4 iron, 4 sulfur cluster binding; electron carrier activity; electron-transferring-flavoprotein dehydrogenase activity; FAD binding; metal ion binding; oxidoreductase activity; oxidoreductase activity, oxidizing metal ions with flavin as acceptor; quinone binding; ubiquinone binding

Biological Process: cellular metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; response to oxidative stress

Disease: Multiple Acyl-coa Dehydrogenase Deficiency