FEBS Letters, volume 595, issue 8, pages 1025-1061

The Diseased Mitoribosome

Ferrari Alberto 1
Delolio Samuel 1, 2
Barrientos A. 1, 3
1
 
Department of Neurology University of Miami Miller School of Medicine FL USA
2
 
Molecular and Cellular Pharmacology Graduate Program University of Miami Miller School of Medicine FL USA
3
 
Department of Biochemistry and Molecular Biology University of Miami Miller School of Medicine FL USA
Publication typeJournal Article
Publication date2020-12-22
Journal: FEBS Letters
Quartile SCImago
Q1
Quartile WOS
Q2
Impact factor3.5
ISSN00145793, 18733468
Biochemistry
Molecular Biology
Cell Biology
Genetics
Structural Biology
Biophysics
Abstract
Mitochondria control life and death in eukaryotic cells. Harboring a unique circular genome, a by‐product of an ancient endosymbiotic event, mitochondria maintains a specialized and evolutionary divergent protein synthesis machinery, the mitoribosome. Mitoribosome biogenesis depends on elements encoded in both the mitochondrial genome (the RNA components) and the nuclear genome (all ribosomal proteins and assembly factors). Recent cryo‐EM structures of mammalian mitoribosomes have illuminated their composition and provided hints regarding their assembly and elusive mitochondrial translation mechanisms. A growing body of literature involves the mitoribosome in inherited primary mitochondrial disorders. Mutations in genes encoding mitoribosomal RNAs, proteins, and assembly factors impede mitoribosome biogenesis, causing protein synthesis defects that lead to respiratory chain failure and mitochondrial disorders such as encephalo‐ and cardiomyopathy, deafness, neuropathy, and developmental delays. In this article, we review the current fundamental understanding of mitoribosome assembly and function, and the clinical landscape of mitochondrial disorders driven by mutations in mitoribosome components and assembly factors, to portray how basic and clinical studies combined help us better understand both mitochondrial biology and medicine.

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GOST Copy
Ferrari A. et al. The Diseased Mitoribosome // FEBS Letters. 2020. Vol. 595. No. 8. pp. 1025-1061.
GOST all authors (up to 50) Copy
Ferrari A., Delolio S., Barrientos A. The Diseased Mitoribosome // FEBS Letters. 2020. Vol. 595. No. 8. pp. 1025-1061.
RIS |
Cite this
RIS Copy
TY - JOUR
DO - 10.1002/1873-3468.14024
UR - https://doi.org/10.1002%2F1873-3468.14024
TI - The Diseased Mitoribosome
T2 - FEBS Letters
AU - Ferrari, Alberto
AU - Delolio, Samuel
AU - Barrientos, A.
PY - 2020
DA - 2020/12/22 00:00:00
PB - Wiley
SP - 1025-1061
IS - 8
VL - 595
PMID - 33314036
SN - 0014-5793
SN - 1873-3468
ER -
BibTex |
Cite this
BibTex Copy
@article{2020_Ferrari
author = {Alberto Ferrari and Samuel Delolio and A. Barrientos},
title = {The Diseased Mitoribosome},
journal = {FEBS Letters},
year = {2020},
volume = {595},
publisher = {Wiley},
month = {dec},
url = {https://doi.org/10.1002%2F1873-3468.14024},
number = {8},
pages = {1025--1061},
doi = {10.1002/1873-3468.14024}
}
MLA
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MLA Copy
Ferrari, Alberto, et al. “The Diseased Mitoribosome.” FEBS Letters, vol. 595, no. 8, Dec. 2020, pp. 1025-1061. https://doi.org/10.1002%2F1873-3468.14024.
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