Mitochondrial protein translation: emerging roles and clinical significance in disease

F Wang, D Zhang, D Zhang, P Li, Y Gao - Frontiers in Cell and …, 2021 - frontiersin.org
Mitochondria are one of the most important organelles in cells. Mitochondria are semi-
autonomous organelles with their own genetic system, and can independently replicate …

The diseased mitoribosome

A Ferrari, S Del'Olio, A Barrientos - FEBS letters, 2021 - Wiley Online Library
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 …

Structural basis of mitochondrial translation

S Aibara, V Singh, A Modelska, A Amunts - elife, 2020 - elifesciences.org
Translation of mitochondrial messenger RNA (mt-mRNA) is performed by distinct
mitoribosomes comprising at least 36 mitochondria-specific proteins. How these …

Structural basis of GTPase-mediated mitochondrial ribosome biogenesis and recycling

HS Hillen, E Lavdovskaia, F Nadler, E Hanitsch… - Nature …, 2021 - nature.com
Ribosome biogenesis requires auxiliary factors to promote folding and assembly of
ribosomal proteins and RNA. Particularly, maturation of the peptidyl transferase center (PTC) …

Structural basis of translation termination, rescue, and recycling in mammalian mitochondria

E Kummer, KN Schubert, T Schoenhut, A Scaiola… - Molecular Cell, 2021 - cell.com
The mitochondrial translation system originates from a bacterial ancestor but has
substantially diverged in the course of evolution. Here, we use single-particle cryo-electron …

A distinct assembly pathway of the human 39S late pre-mitoribosome

J Cheng, O Berninghausen, R Beckmann - Nature communications, 2021 - nature.com
Assembly of the mitoribosome is largely enigmatic and involves numerous assembly factors.
Little is known about their function and the architectural transitions of the pre-ribosomal …

Ratchet, swivel, tilt and roll: a complete description of subunit rotation in the ribosome

A Hassan, S Byju, FC Freitas, C Roc… - Nucleic acids …, 2023 - academic.oup.com
Protein synthesis by the ribosome requires large-scale rearrangements of the 'small'subunit
(SSU;∼ 1 MDa), including inter-and intra-subunit rotational motions. However, with nearly …

Dual function of GTPBP6 in biogenesis and recycling of human mitochondrial ribosomes

E Lavdovskaia, K Denks, F Nadler… - Nucleic Acids …, 2020 - academic.oup.com
Translation and ribosome biogenesis in mitochondria require auxiliary factors that ensure
rapid and accurate synthesis of mitochondrial proteins. Defects in translation are associated …

Quantifying constraint in the human mitochondrial genome

NJ Lake, K Ma, W Liu, SL Battle, KM Laricchia, G Tiao… - Nature, 2024 - nature.com
Mitochondrial DNA (mtDNA) has an important yet often overlooked role in health and
disease. Constraint models quantify the removal of deleterious variation from the population …