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Molecular Mechanism of Mitochondrial Respiratory Chain Assembly and Its Relation to Mitochondrial Diseases by Mukherjee, Soumyajit; Ghosh, Alok is a Biochemistry, Genetics and Molecular Biology article available to read on EtoBox.

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The mitochondrial respiratory chain (MRC) is comprised of ~92 nuclear and mitochondrial DNA-encoded protein subunits that are organized into five different multi-subunit respiratory complexes. These complexes produce 90% of the ATP required for cell sustenance. Specific sets of subunits are assembled in a modular or non-modular fashion to construct the MRC complexes. The complete assembly process is gradually chaperoned by a myriad of assembly factors that must coordinate with several other prosthetic groups to reach maturity, makingthe entire processextensively complicated. Further, the individual respiratory complexes can be integrated intovarious giant super-complexes whose functional roles have yet to be explored. Mutations in the MRC subunits and in the related assembly factors often give rise to defects in the proper assembly of the respiratory chain, which then manifests as a group of disorders called mitochondrial diseases, the most common inborn errors of metabolism. This review summarizes the current understanding of the biogenesis of individual MRC complexes and super-complexes, and explores how mutations in the different subunits and assembly factors contribute to mitoc

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Author

Mukherjee, Soumyajit; Ghosh, Alok

Publisher

Elsevier Science; Elsevier ; Elsevier BV (ISSN 1567-7249)

Published

2020

Language

EN

Field

Biochemistry, Genetics and Molecular Biology (Life Sciences)