NDUFA5 Human

NDUFA5 is one of the many subunits that make up Complex I, the largest and most intricate enzyme of the electron transport chain . The protein encoded by NDUFA5 localizes to the inner mitochondrial membrane, where it plays a crucial role in the electron transfer process . Specifically, it aids in the transfer of electrons from NADH to ubiquinone, facilitating the reduction of ubiquinone to ubiquinol .

The structure of Complex I is L-shaped, with a long, hydrophobic transmembrane domain and a hydrophilic domain that includes all the known redox centers and the NADH binding site . NDUFA5 is one of the hydrophobic subunits that form the transmembrane region of Complex I .

The proper functioning of Complex I is vital for the normal operation of cellular respiration. Mutations in the subunits of Complex I, including NDUFA5, can lead to a variety of inherited neuromuscular and metabolic disorders . These defects can result in conditions such as mitochondrial Complex I deficiency, which is associated with a range of pathological processes, including ischemia/reperfusion damage, Parkinson’s disease, and other neurodegenerative disorders .

Recombinant NDUFA5 refers to the protein produced through recombinant DNA technology, which allows for the expression of the human NDUFA5 gene in a host organism, such as bacteria or yeast. This technology enables the production of large quantities of the protein for research and therapeutic purposes. Recombinant NDUFA5 is used in various studies to understand its role in mitochondrial function and its involvement in disease processes.

Research on NDUFA5 and its role in Complex I has significant implications for understanding mitochondrial diseases and developing potential therapies. By studying the structure and function of recombinant NDUFA5, scientists can gain insights into the mechanisms underlying mitochondrial dysfunction and identify potential targets for therapeutic intervention.