Modular biogenesis of mitochondrial respiratory complexes

Author(s)

Barros, Mario H. ^[1] ; McStay, Gavin P. ^[2]

Affiliation

^[1] Univ Sao Paulo, Dept Microbiol, Inst Ciencias Biomed, Sao Paulo - Brazil

^[2] Staffordshire Univ, Dept Biol Sci, Stoke On Trent, Staffs - England

• Document type: Review article
• Source: MITOCHONDRION; v. 50, p. 94-114, JAN 2020.
• Web of Science Citations: 0

Abstract

Mitochondrial function relies on the activity of oxidative phosphorylation to synthesise ATP and generate an electrochemical gradient across the inner mitochondrial membrane. These coupled processes are mediated by five multi-subunit complexes that reside in this inner membrane. These complexes are the product of both nuclear and mitochondrial gene products. Defects in the function or assembly of these complexes can lead to mitochondrial diseases due to deficits in energy production and mitochondrial functions. Appropriate biogenesis and function are mediated by a complex number of assembly factors that promote maturation of specific complex subunits to form the active oxidative phosphorylation complex. The understanding of the biogenesis of each complex has been informed by studies in both simple eukaryotes such as Saccharomyces cerevisiae and human patients with mitochondrial diseases. These studies reveal each complex assembles through a pathway using specific subunits and assembly factors to form kinetically distinct but related assembly modules. The current understanding of these complexes has embraced the revolutions in genomics and proteomics to further our knowledge on the impact of mitochondrial biology in genetics, medicine, and evolution. (AU)

• FAPESP's process: 19/05232-3 - Co-regulatory studies onto mitochondrial ribosome biogenesis and cytochrome oxidase assembly
• Grantee: Mario Henrique de Barros
• Support Opportunities: Research Grants - Visiting Researcher Grant - International