NDUFAF3 Activators

NDUFAF3, standing for NADH:ubiquinone oxidoreductase complex assembly factor 3, is a protein that is instrumental in the assembly of mitochondrial complex I, also known as NADH:ubiquinone oxidoreductase. Complex I is the largest and first enzyme complex in the mitochondrial electron transport chain (ETC), which is a critical component of oxidative phosphorylation, the primary pathway through which eukaryotic cells convert nutrients into energy in the form of ATP.

The assembly and maintenance of complex I are intricate processes that require the coordination of various assembly factors. NDUFAF3 is one of these factors and is involved in the early stages of the assembly process. It plays a fundamental role in the assembly of the membrane arm and the stabilization of intermediate complex I assembly modules. Mutations in the NDUFAF3 gene have been associated with mitochondrial diseases, highlighting its essential role in proper cellular function and energy metabolism.NDUFAF3 functions within the mitochondria, where it interacts with other proteins and assembly factors to ensure the correct assembly and integration of the various subunits that constitute complex I. Complex I itself is composed of over 40 different subunits and is responsible for transferring electrons from NADH to coenzyme Q10 (ubiquinone) in the ETC, a process that contributes to the creation of a proton gradient across the mitochondrial inner membrane. This gradient is ultimately used to drive the synthesis of ATP by ATP synthase.