Assembly factors of human mitochondrial complex I and their defects in disease

✍ Scribed by Matthew Mckenzie; Michael T. Ryan

• Publisher: John Wiley and Sons
• Year: 2010
• Tongue: English
• Weight: 159 KB
• Volume: 62
• Category: Article
• ISSN: 1521-6543
• DOI: 10.1002/iub.335

✦ Synopsis

Abstract

NADH‐ubiquinone oxidoreductase (complex I) is a large, multimeric enzyme complex involved in the generation of ATP by oxidative phosphorylation. Complex I is comprised of 45 subunits which must be assembled together in a coordinated process to form the mature holoenzyme. In recent years, much progress has been made into understanding how complex I is assembled and the work provides potential insights into the biogenesis of other multisubunit membrane complexes. For complex I assembly to proceed effectively, a group of proteins termed “assembly factors” are required. A number of these assembly factors have now been identified and characterized; however, their exact roles in complex I biogenesis are not yet fully understood. This review summarizes the current model of human complex I assembly and the roles played by different assembly factors at early, mid, and late assembly stages. Defects in assembly factors which disrupt complex I assembly and contribute to human disease pathogenesis will also be discussed. © 2010 IUBMB IUBMB Life 62(7): 497–502, 2010