NDUFA11

NDUFA11 (NADH:Ubiquinone Oxidoreductase Subunit A11): NDUFA11 is a component of the mitochondrial complex I, the largest and first enzyme complex in the mitochondrial electron transport chain (ETC), crucial for cellular energy production. It plays a vital role in the assembly, stability, and function of complex I, facilitating the transfer of electrons from NADH to ubiquinone, thereby contributing to the generation of the proton gradient used to produce ATP through oxidative phosphorylation. Given its essential role in cellular metabolism and energy production, dysfunctions or mutations in NDUFA11 can lead to mitochondrial diseases and contribute to a range of metabolic and degenerative disorders, including neuromuscular diseases and complex I deficiency syndromes. The importance of NDUFA11 in mitochondrial function underscores its potential as a target for therapeutic intervention in diseases arising from mitochondrial dysfunction.