NDUFS4
Description
NDUFS4, also known as NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, is a crucial component of Complex I, the first enzyme in the electron transport chain (ETC). The ETC is a series of protein complexes located within the inner mitochondrial membrane, responsible for generating ATP, the primary energy source of cells. NDUFS4 is a small, hydrophobic protein that plays a critical role in electron transfer within Complex I. It acts as a scaffold, holding together various subunits and facilitating the movement of electrons from NADH to coenzyme Q. This process is vital for cellular respiration and ultimately, the survival of the organism.
Associated Diseases
Did you know?
Mutations in the NDUFS4 gene are associated with a rare genetic disorder called Leigh Syndrome, a severe neurological condition characterized by progressive brain damage.