FEBS Letters, volume 595, issue 8, pages 1025-1061
The Diseased Mitoribosome
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 type: Journal Article
Publication date: 2020-12-22
Journal:
FEBS Letters
Quartile SCImago
Q1
Quartile WOS
Q2
Impact factor: 3.5
ISSN: 00145793, 18733468
PubMed ID:
33314036
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.
Citations by journals
1
2
3
|
|
Methods in Molecular Biology
|
Methods in Molecular Biology
3 publications, 7.14%
|
International Journal of Molecular Sciences
|
International Journal of Molecular Sciences
2 publications, 4.76%
|
Nature Communications
|
Nature Communications
2 publications, 4.76%
|
RNA Biology
|
RNA Biology
2 publications, 4.76%
|
Nucleic Acids Research
|
Nucleic Acids Research
2 publications, 4.76%
|
Biomolecules
|
Biomolecules
1 publication, 2.38%
|
Genes
|
Genes
1 publication, 2.38%
|
Cells
|
Cells
1 publication, 2.38%
|
Biomedicines
|
Biomedicines
1 publication, 2.38%
|
Frontiers in Cell and Developmental Biology
|
Frontiers in Cell and Developmental Biology
1 publication, 2.38%
|
Frontiers in Pharmacology
|
Frontiers in Pharmacology
1 publication, 2.38%
|
Nature Reviews Molecular Cell Biology
|
Nature Reviews Molecular Cell Biology
1 publication, 2.38%
|
iScience
|
iScience
1 publication, 2.38%
|
Biochimie
|
Biochimie
1 publication, 2.38%
|
Trends in Parasitology
|
Trends in Parasitology
1 publication, 2.38%
|
Trends in Cell Biology
|
Trends in Cell Biology
1 publication, 2.38%
|
Mitochondrion
|
Mitochondrion
1 publication, 2.38%
|
Human Genetics
|
Human Genetics
1 publication, 2.38%
|
FEBS Letters
|
FEBS Letters
1 publication, 2.38%
|
Yeast
|
Yeast
1 publication, 2.38%
|
eLife
|
eLife
1 publication, 2.38%
|
Biology
|
Biology
1 publication, 2.38%
|
EMBO Reports
|
EMBO Reports
1 publication, 2.38%
|
Newborn
|
Newborn, 1, 2.38%
Newborn
1 publication, 2.38%
|
Human Molecular Genetics
|
Human Molecular Genetics
1 publication, 2.38%
|
Frontiers in Molecular Biosciences
|
Frontiers in Molecular Biosciences
1 publication, 2.38%
|
Current Pediatric Reviews
|
Current Pediatric Reviews
1 publication, 2.38%
|
Environmental Research
|
Environmental Research
1 publication, 2.38%
|
IUBMB Life
|
IUBMB Life
1 publication, 2.38%
|
1
2
3
|
Citations by publishers
1
2
3
4
5
6
7
|
|
Multidisciplinary Digital Publishing Institute (MDPI)
|
Multidisciplinary Digital Publishing Institute (MDPI)
7 publications, 16.67%
|
Springer Nature
|
Springer Nature
7 publications, 16.67%
|
Elsevier
|
Elsevier
6 publications, 14.29%
|
Frontiers Media S.A.
|
Frontiers Media S.A.
3 publications, 7.14%
|
Wiley
|
Wiley
3 publications, 7.14%
|
Oxford University Press
|
Oxford University Press
3 publications, 7.14%
|
Taylor & Francis
|
Taylor & Francis
2 publications, 4.76%
|
eLife Sciences Publications
|
eLife Sciences Publications
1 publication, 2.38%
|
EMBO press
|
EMBO press
1 publication, 2.38%
|
Jaypee Brothers Medical Publishing
|
Jaypee Brothers Medical Publishing, 1, 2.38%
Jaypee Brothers Medical Publishing
1 publication, 2.38%
|
Bentham Science
|
Bentham Science
1 publication, 2.38%
|
1
2
3
4
5
6
7
|
- We do not take into account publications that without a DOI.
- Statistics recalculated only for publications connected to researchers, organizations and labs registered on the platform.
- Statistics recalculated weekly.
{"yearsCitations":{"type":"bar","data":{"show":true,"labels":[2021,2022,2023,2024],"ids":[0,0,0,0],"codes":[0,0,0,0],"imageUrls":["","","",""],"datasets":[{"label":"Citations number","data":[14,7,20,1],"backgroundColor":["#3B82F6","#3B82F6","#3B82F6","#3B82F6"],"percentage":["33.33","16.67","47.62","2.38"],"barThickness":null}]},"options":{"indexAxis":"x","maintainAspectRatio":true,"scales":{"y":{"ticks":{"precision":0,"autoSkip":false,"font":{"family":"Montserrat"},"color":"#000000"}},"x":{"ticks":{"stepSize":1,"precision":0,"font":{"family":"Montserrat"},"color":"#000000"}}},"plugins":{"legend":{"position":"top","labels":{"font":{"family":"Montserrat"},"color":"#000000"}},"title":{"display":true,"text":"Citations per year","font":{"size":24,"family":"Montserrat","weight":600},"color":"#000000"}}}},"journals":{"type":"bar","data":{"show":true,"labels":["Methods in Molecular Biology","International Journal of Molecular Sciences","Nature Communications","RNA Biology","Nucleic Acids Research","Biomolecules","Genes","Cells","Biomedicines","Frontiers in Cell and Developmental Biology","Frontiers in Pharmacology","Nature Reviews Molecular Cell Biology","iScience","Biochimie","Trends in Parasitology","Trends in Cell Biology","Mitochondrion","Human Genetics","FEBS Letters","Yeast","eLife","Biology","EMBO Reports","Newborn","Human Molecular Genetics","Frontiers in Molecular Biosciences","Current Pediatric Reviews","Environmental Research","IUBMB Life"],"ids":[3516,14627,3231,7613,23904,13780,8879,25293,24421,12128,9788,10207,6073,4794,18145,4177,18142,18819,405,11674,24138,24144,2390,41273,4947,3133,13197,5708,6564],"codes":[0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0],"imageUrls":["\/storage\/images\/resized\/voXLqlsvTwv5p3iMQ8Dhs95nqB4AXOG7Taj7G4ra_medium.webp","\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/voXLqlsvTwv5p3iMQ8Dhs95nqB4AXOG7Taj7G4ra_medium.webp","\/storage\/images\/resized\/5YZtvLvkPZuc2JHOaZsjCvGSHFCuC3drUwN3YAc5_medium.webp","\/storage\/images\/resized\/yNSijlgQghQF53VZuyFLA30CKDe4j3HK74Vtpnxa_medium.webp","\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/4QWA67eqfcfyOiA8Wk7YnqroHFqQbTsmDJUYTCTg_medium.webp","\/storage\/images\/resized\/4QWA67eqfcfyOiA8Wk7YnqroHFqQbTsmDJUYTCTg_medium.webp","\/storage\/images\/resized\/voXLqlsvTwv5p3iMQ8Dhs95nqB4AXOG7Taj7G4ra_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/voXLqlsvTwv5p3iMQ8Dhs95nqB4AXOG7Taj7G4ra_medium.webp","\/storage\/images\/resized\/bRyGpdm98BkAUYiK1YFNpl5Z7hPu6Gd87gbIeuG3_medium.webp","\/storage\/images\/resized\/bRyGpdm98BkAUYiK1YFNpl5Z7hPu6Gd87gbIeuG3_medium.webp","\/storage\/images\/resized\/eIZgkq1MRZyJ3hBkdupnUUv7wgB6zCl9D99SlPN5_medium.webp","\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/1UiI5HycPsvulDeXOySNp0OuaMMmIKfPnsdKPyH0_medium.webp","","\/storage\/images\/resized\/yNSijlgQghQF53VZuyFLA30CKDe4j3HK74Vtpnxa_medium.webp","\/storage\/images\/resized\/4QWA67eqfcfyOiA8Wk7YnqroHFqQbTsmDJUYTCTg_medium.webp","\/storage\/images\/resized\/doUaFUZdxUEQjLi1TwZjGHi8HXYNWWSk04dSC6Xh_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/bRyGpdm98BkAUYiK1YFNpl5Z7hPu6Gd87gbIeuG3_medium.webp"],"datasets":[{"label":"","data":[3,2,2,2,2,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1],"backgroundColor":["#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6"],"percentage":[7.14,4.76,4.76,4.76,4.76,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38,2.38],"barThickness":13}]},"options":{"indexAxis":"y","maintainAspectRatio":false,"scales":{"y":{"ticks":{"precision":0,"autoSkip":false,"font":{"family":"Montserrat"},"color":"#000000"}},"x":{"ticks":{"stepSize":null,"precision":0,"font":{"family":"Montserrat"},"color":"#000000"}}},"plugins":{"legend":{"position":"top","labels":{"font":{"family":"Montserrat"},"color":"#000000"}},"title":{"display":true,"text":"Journals","font":{"size":24,"family":"Montserrat","weight":600},"color":"#000000"}}}},"publishers":{"type":"bar","data":{"show":true,"labels":["Multidisciplinary Digital Publishing Institute (MDPI)","Springer Nature","Elsevier","Frontiers Media S.A.","Wiley","Oxford University Press","Taylor & Francis","eLife Sciences Publications","EMBO press","Jaypee Brothers Medical Publishing","Bentham Science"],"ids":[202,8,17,208,11,19,18,6627,9415,7373,39],"codes":[0,0,0,0,0,0,0,0,0,0,0],"imageUrls":["\/storage\/images\/resized\/MjH1ITP7lMYGxeqUZfkt2BnVLgjkk413jwBV97XX_medium.webp","\/storage\/images\/resized\/voXLqlsvTwv5p3iMQ8Dhs95nqB4AXOG7Taj7G4ra_medium.webp","\/storage\/images\/resized\/GDnYOu1UpMMfMMRV6Aqle4H0YLLsraeD9IP9qScG_medium.webp","\/storage\/images\/resized\/4QWA67eqfcfyOiA8Wk7YnqroHFqQbTsmDJUYTCTg_medium.webp","\/storage\/images\/resized\/bRyGpdm98BkAUYiK1YFNpl5Z7hPu6Gd87gbIeuG3_medium.webp","\/storage\/images\/resized\/yNSijlgQghQF53VZuyFLA30CKDe4j3HK74Vtpnxa_medium.webp","\/storage\/images\/resized\/5YZtvLvkPZuc2JHOaZsjCvGSHFCuC3drUwN3YAc5_medium.webp","\/storage\/images\/resized\/eIZgkq1MRZyJ3hBkdupnUUv7wgB6zCl9D99SlPN5_medium.webp","\/storage\/images\/resized\/1UiI5HycPsvulDeXOySNp0OuaMMmIKfPnsdKPyH0_medium.webp","","\/storage\/images\/resized\/doUaFUZdxUEQjLi1TwZjGHi8HXYNWWSk04dSC6Xh_medium.webp"],"datasets":[{"label":"","data":[7,7,6,3,3,3,2,1,1,1,1],"backgroundColor":["#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6","#3B82F6"],"percentage":[16.67,16.67,14.29,7.14,7.14,7.14,4.76,2.38,2.38,2.38,2.38],"barThickness":13}]},"options":{"indexAxis":"y","maintainAspectRatio":false,"scales":{"y":{"ticks":{"precision":0,"autoSkip":false,"font":{"family":"Montserrat"},"color":"#000000"}},"x":{"ticks":{"stepSize":null,"precision":0,"font":{"family":"Montserrat"},"color":"#000000"}}},"plugins":{"legend":{"position":"top","labels":{"font":{"family":"Montserrat"},"color":"#000000"}},"title":{"display":true,"text":"Publishers","font":{"size":24,"family":"Montserrat","weight":600},"color":"#000000"}}}}}
Metrics
Cite this
GOST |
RIS |
BibTex |
MLA
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 -
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}
}
Cite this
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.