ETFB Human
Description
The ETFB is purified by proprietary chromatographic techniques.
Product Specs
KVEREIDGGL ETLRLKLPAV VTADLRLNEP RYATLPNIMK AKKKKIEVIK PGDLGVDLTS KLSVISVEDP PQRTAGVKVE TTEDLVAKLK EIGRI.
Product Science Overview
ETFB is a heterodimeric protein composed of an alpha and beta subunit. The beta subunit, which is the focus here, contains an FAD (flavin adenine dinucleotide) cofactor and an AMP (adenosine monophosphate) molecule. These molecules are crucial for the protein’s function in electron transfer . The ETF complex accepts electrons from various mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, and transfers them to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase .
ETFB is involved in the metabolism of fatty acids and amino acids. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. This process is vital for normal mitochondrial fatty acid oxidation and amino acid metabolism . Deficiencies in ETFB can lead to metabolic disorders such as Multiple Acyl-CoA Dehydrogenase Deficiency (MADD), also known as glutaric acidemia type II .
Recombinant ETFB is produced using recombinant DNA technology, which involves inserting the ETFB gene into a suitable expression system, such as E. coli, to produce the protein in large quantities. The recombinant protein is then purified using chromatographic techniques . This recombinant form is used in research to study the protein’s structure, function, and role in metabolic diseases.
Mutations in the ETFB gene can lead to severe metabolic disorders. For instance, MADD is characterized by the accumulation of organic acids and fatty acid metabolites in the body, leading to symptoms such as muscle weakness, hypoglycemia, and metabolic acidosis . Understanding the structure and function of ETFB is crucial for developing therapeutic strategies for these conditions.
Research on ETFB has provided insights into its role in mitochondrial function and its involvement in metabolic diseases. Studies have shown that ETFB binds an AMP molecule that likely has a structural role . Additionally, the three-dimensional structure of human ETFB has been resolved, providing a detailed understanding of its function at the molecular level .
