Insights into mitoribosomal biogenesis from recent structural studies

A Khawaja, M Cipullo, A Krüger, J Rorbach - Trends in Biochemical …, 2023 - cell.com
The mitochondrial ribosome (mitoribosome) is a multicomponent machine that has unique
structural features. Biogenesis of the human mitoribosome includes correct maturation and …

Schizosaccharomyces pombe as a fundamental model for research on mitochondrial gene expression: Progress, achievements and outlooks

N Dinh, N Bonnefoy - IUBMB life, 2024 - Wiley Online Library
Schizosaccharomyces pombe (fission yeast) is an attractive model for mitochondrial
research. The organism resembles human cells in terms of mitochondrial inheritance …

BOLA3 and NFU1 link mitoribosome iron–sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome

H Zhong, A Janer, O Khalimonchuk… - Nucleic acids …, 2023 - academic.oup.com
The human mitochondrial ribosome contains three [2Fe–2S] clusters whose assembly
pathway, role, and implications for mitochondrial and metabolic diseases are unknown …

[HTML][HTML] Enzymes of the crotonase superfamily: Diverse assembly and diverse function

S Dalwani, RK Wierenga - Current Opinion in Structural Biology, 2023 - Elsevier
The crotonase fold is generated by a framework of four repeats of a ββα-unit, extended by
two helical regions. The active site of crotonase superfamily (CS) enzymes is located at the …

Structural insights into the specific recognition of mitochondrial ribosome-binding factor hsRBFA and 12 S rRNA by methyltransferase METTL15

M Lv, W Zhou, Y Hao, F Li, H Zhang, X Yao, Y Shi… - Cell Discovery, 2024 - nature.com
Mitochondrial rRNA modifications are essential for mitoribosome assembly and its proper
function. The m4C methyltransferase METTL15 maintains mitochondrial homeostasis by …

GTPase Era at the heart of ribosome assembly

C Gruffaz, A Smirnov - Frontiers in Molecular Biosciences, 2023 - frontiersin.org
Ribosome biogenesis is a key process in all organisms. It relies on coordinated work of
multiple proteins and RNAs, including an array of assembly factors. Among them, the …

Miniature RNAs are embedded in an exceptionally protein-rich mitoribosome via an elaborate assembly pathway

M Valach, C Benz, LC Aguilar, O Gahura… - Nucleic Acids …, 2023 - academic.oup.com
The mitochondrial ribosome (mitoribosome) has diverged drastically from its evolutionary
progenitor, the bacterial ribosome. Structural and compositional diversity is particularly …

GTPBP8 plays a role in mitoribosome formation in human mitochondria

M Cipullo, G Valentín Gesé, S Gopalakrishna… - Nature …, 2024 - nature.com
Mitochondrial gene expression relies on mitoribosomes to translate mitochondrial mRNAs.
The biogenesis of mitoribosomes is an intricate process involving multiple assembly factors …

Mitoribosome structure with cofactors and modifications reveals mechanism of ligand binding and interactions with L1 stalk

V Singh, Y Itoh, S Del'Olio, A Hassan… - Nature …, 2024 - nature.com
The mitoribosome translates mitochondrial mRNAs and regulates energy conversion that is
a signature of aerobic life forms. We present a 2.2 Å resolution structure of human …

Let's make it clear: systematic exploration of mitochondrial DNA–and RNA–protein complexes by complexome profiling

A Potter, A Cabrera-Orefice… - Nucleic Acids …, 2023 - academic.oup.com
Complexome profiling (CP) is a powerful tool for systematic investigation of protein
interactors that has been primarily applied to study the composition and dynamics of …