NDUFS4 Human
Description
The NDUFS4 is purified by proprietary chromatographic techniques.
Product Specs
Product Science Overview
Histidine NADH Dehydrogenase Fe-S Protein 4, also known as NDUFS4, is a crucial component of the mitochondrial respiratory chain complex I. This protein plays a significant role in cellular respiration, which is essential for energy production in eukaryotic cells. The human recombinant form of this protein is produced using recombinant DNA technology, which allows for the expression of the protein in a host organism, typically Escherichia coli.
NDUFS4 is a non-glycosylated polypeptide chain consisting of 134 amino acids, with a molecular mass of approximately 15.5 kDa . The protein contains iron-sulfur (Fe-S) clusters, which are essential for its function. These clusters are prosthetic groups that facilitate electron transfer within the protein. The most common Fe-S clusters found in NDUFS4 are [2Fe-2S] and [4Fe-4S], which are typically coordinated by cysteine residues .
The primary function of NDUFS4 is to participate in the electron transport chain (ETC) within mitochondria. It is a subunit of Complex I (NADH:ubiquinone oxidoreductase), which is the first enzyme complex in the ETC. Complex I catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q10), a process that is coupled with the translocation of protons across the mitochondrial inner membrane. This proton gradient is then used to generate ATP, the cell’s primary energy currency .
Mutations in the NDUFS4 gene can lead to mitochondrial complex I deficiency, a condition that affects the oxidative phosphorylation pathway. This deficiency can result in a range of clinical manifestations, including Leigh syndrome, a severe neurological disorder characterized by progressive loss of mental and movement abilities . Understanding the structure and function of NDUFS4 is crucial for developing therapeutic strategies for these mitochondrial disorders.
The human recombinant form of NDUFS4 is produced by inserting the gene encoding the protein into a plasmid vector, which is then introduced into a host organism such as E. coli. The host cells express the protein, which can be purified and used for various research and therapeutic applications. Recombinant NDUFS4 is valuable for studying the protein’s structure, function, and role in disease, as well as for developing potential treatments .
