ETFDH blocking peptide

ETFDH Peptide - C-terminal region

Cat. Num. AAA3239563

• Gene Names: ETFDH; MADD; ETFQO
• Reactivity: Human
• Applications: Western Blot
• Synonyms: ETFDH; ETFDH Peptide - C-terminal region; ETFDH blocking peptide

• Reactivity: Human
• Form/Format: Lyophilized powder
• Sequence: KTIGLHVTEYEDNLKNSWVWKELYSVRNIRPSCHGVLGVYGGMIYTGIFY
• Sequence Length: 617

• Applicable Applications for ETFDH blocking peptide: Western Blot (WB)
• Preparation and Storage: Add 100ul of sterile PBS. Final peptide concentration is 1 mg/ml in PBS. For longer periods of storage, store at -20 degree C. Avoid repeat freeze-thaw cycles.

Related Product Information for ETFDH blocking peptide

This is a synthetic peptide designed for use in combination with anti-ETFDH Antibody, made

Target Description: Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane. Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type II glutaricacidemia

Product Categories/Family for ETFDH blocking peptide

Peptide

NCBI and Uniprot Product Information

• NCBI GI #: 119703746
• NCBI GeneID: 2110
• NCBI Accession #: NP_004444
• NCBI GenBank Nucleotide #: NM_004453
• UniProt Accession #: Q16134
• Molecular Weight: 65kDa
• 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
• 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: Mitochondrial; EC 1.5.5.1; Membrane protein, integral; Oxidoreductase

Chromosomal Location of Human Ortholog: 4q32-q35

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

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

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

Disease: Multiple Acyl-coa Dehydrogenase Deficiency