Journal of Physiology, volume 599, issue 14, pages 3449-3462

Fidelity and coordination of mitochondrial protein synthesis in health and disease

Rudler Danielle L. 1, 2, 3
Hughes Laetitia A 1, 2, 3
Viola Helena 4
Hool Livia C. 4, 5
Rackham Oliver 1, 2, 6, 7, 8
Filipovska Aleksandra 1, 2, 3, 8, 9
1
 
Harry Perkins Institute of Medical Research QEII Medical Centre Nedlands Western Australia 6009 Australia
2
 
ARC Centre of Excellence in Synthetic Biology QEII Medical Centre Nedlands Western Australia 6009 Australia
3
 
Centre for Medical Research, The University of Western Australia QEII Medical Centre Nedlands Western Australia 6009 Australia
4
 
School of Human Sciences University of Western Australia 35 Stirling Highway Nedlands Western Australia 6009 Australia
5
 
Victor Chang Cardiac Research Institute Sydney NSW Australia
6
 
School of Pharmacy and Biomedical Sciences Curtin University Bentley Western Australia 6102 Australia
7
 
Curtin Health Innovation Research Institute Curtin University Bentley Western Australia 6102 Australia
8
 
Telethon Kids Institute, Northern Entrance Perth Children's Hospital 15 Hospital Avenue Nedlands Western Australia Australia
9
 
School of Molecular Sciences University of Western Australia Crawley Western Australia 6009 Australia
Publication typeJournal Article
Publication date2020-08-15
Quartile SCImago
Q1
Quartile WOS
Q1
Impact factor5.5
ISSN00223751, 14697793
Physiology
Abstract
The evolutionary acquisition of mitochondria has given rise to the diversity of eukaryotic life. Mitochondria have retained their ancestral α‐proteobacterial traits through the maintenance of double membranes and their own circular genome. Their genome varies in size from very large in plants to the smallest in animals and their parasites. The mitochondrial genome encodes essential genes for protein synthesis and has to coordinate its expression with the nuclear genome from which it sources most of the proteins required for mitochondrial biogenesis and function. The mitochondrial protein synthesis machinery is unique because it is encoded by both the nuclear and mitochondrial genomes thereby requiring tight regulation to produce the respiratory complexes that drive oxidative phosphorylation for energy production. The fidelity and coordination of mitochondrial protein synthesis are essential for ATP production. Here we compare and contrast the mitochondrial translation mechanisms in mammals and fungi to bacteria and reveal that their diverse regulation can have unusual impacts on the health and disease of these organisms. We highlight that in mammals the rate of protein synthesis is more important than the fidelity of translation, enabling coordinated biogenesis of the mitochondrial respiratory chain with respiratory chain proteins synthesised by cytoplasmic ribosomes. Changes in mitochondrial protein fidelity can trigger the activation of the diverse cellular signalling networks in fungi and mammals to combat dysfunction in energy conservation. The physiological consequences of altered fidelity of protein synthesis can range from liver regeneration to the onset and development of cardiomyopathy.

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GOST Copy
Rudler D. L. et al. Fidelity and coordination of mitochondrial protein synthesis in health and disease // Journal of Physiology. 2020. Vol. 599. No. 14. pp. 3449-3462.
GOST all authors (up to 50) Copy
Rudler D. L., Hughes L. A., Viola H., Hool L. C., Rackham O., Filipovska A. Fidelity and coordination of mitochondrial protein synthesis in health and disease // Journal of Physiology. 2020. Vol. 599. No. 14. pp. 3449-3462.
RIS |
Cite this
RIS Copy
TY - JOUR
DO - 10.1113/JP280359
UR - https://doi.org/10.1113%2FJP280359
TI - Fidelity and coordination of mitochondrial protein synthesis in health and disease
T2 - Journal of Physiology
AU - Viola, Helena
AU - Rackham, Oliver
AU - Filipovska, Aleksandra
AU - Rudler, Danielle L.
AU - Hughes, Laetitia A
AU - Hool, Livia C.
PY - 2020
DA - 2020/08/15 00:00:00
PB - Wiley
SP - 3449-3462
IS - 14
VL - 599
SN - 0022-3751
SN - 1469-7793
ER -
BibTex |
Cite this
BibTex Copy
@article{2020_Rudler,
author = {Helena Viola and Oliver Rackham and Aleksandra Filipovska and Danielle L. Rudler and Laetitia A Hughes and Livia C. Hool},
title = {Fidelity and coordination of mitochondrial protein synthesis in health and disease},
journal = {Journal of Physiology},
year = {2020},
volume = {599},
publisher = {Wiley},
month = {aug},
url = {https://doi.org/10.1113%2FJP280359},
number = {14},
pages = {3449--3462},
doi = {10.1113/JP280359}
}
MLA
Cite this
MLA Copy
Rudler, Danielle L., et al. “Fidelity and coordination of mitochondrial protein synthesis in health and disease.” Journal of Physiology, vol. 599, no. 14, Aug. 2020, pp. 3449-3462. https://doi.org/10.1113%2FJP280359.
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