Mitochondrial protein transport: Versatility of translocases and mechanisms

JD Busch, LF Fielden, N Pfanner, N Wiedemann - Molecular Cell, 2023 - cell.com
Biogenesis of mitochondria requires the import of approximately 1,000 different precursor
proteins into and across the mitochondrial membranes. Mitochondria exhibit a wide variety …

Mitochondrial proteins: from biogenesis to functional networks

N Pfanner, B Warscheid, N Wiedemann - Nature reviews Molecular cell …, 2019 - nature.com
Mitochondria are essential for the viability of eukaryotic cells as they perform crucial
functions in bioenergetics, metabolism and signalling and have been associated with …

A genome-wide atlas of co-essential modules assigns function to uncharacterized genes

M Wainberg, RA Kamber, A Balsubramani… - Nature …, 2021 - nature.com
A central question in the post-genomic era is how genes interact to form biological
pathways. Measurements of gene dependency across hundreds of cell lines have been …

The ADP/ATP translocase drives mitophagy independent of nucleotide exchange

A Hoshino, W Wang, S Wada, C McDermott-Roe… - Nature, 2019 - nature.com
Mitochondrial homeostasis depends on mitophagy, the programmed degradation of
mitochondria. Only a few proteins are known to participate in mitophagy. Here we develop a …

Mitochondrial protein import dysfunction: mitochondrial disease, neurodegenerative disease and cancer

CS Palmer, AJ Anderson, D Stojanovski - FEBS letters, 2021 - Wiley Online Library
The majority of proteins localised to mitochondria are encoded by the nuclear genome, with
approximately 1500 proteins imported into mammalian mitochondria. Dysfunction in this …

Acylglycerol kinase mutated in Sengers syndrome is a subunit of the TIM22 protein translocase in mitochondria

M Vukotic, H Nolte, T König, S Saita, M Ananjew… - Molecular cell, 2017 - cell.com
Mutations in mitochondrial acylglycerol kinase (AGK) cause Sengers syndrome, which is
characterized by cataracts, hypertrophic cardiomyopathy, and skeletal myopathy. AGK …

Dissecting the roles of mitochondrial complex I intermediate assembly complex factors in the biogenesis of complex I

LE Formosa, L Muellner-Wong, B Reljic, AJ Sharpe… - Cell reports, 2020 - cell.com
Mitochondrial complex I harbors 7 mitochondrial and 38 nuclear-encoded subunits. Its
biogenesis requires the assembly and integration of distinct intermediate modules, mediated …

Sengers syndrome-associated mitochondrial acylglycerol kinase is a subunit of the human TIM22 protein import complex

Y Kang, DA Stroud, MJ Baker, DP De Souza… - Molecular cell, 2017 - cell.com
Acylglycerol kinase (AGK) is a mitochondrial lipid kinase that catalyzes the phosphorylation
of monoacylglycerol and diacylglycerol to lysophosphatidic acid and phosphatidic acid …

Mitochondrial protein transport in health and disease

Y Kang, LF Fielden, D Stojanovski - Seminars in cell & developmental …, 2018 - Elsevier
Mitochondria are fundamental structures that fulfil important and diverse functions within
cells, including cellular respiration and iron-sulfur cluster biogenesis. Mitochondrial function …

Mitochondrial protein import clogging as a mechanism of disease

LP Coyne, X Wang, J Song, E de Jong, K Schneider… - Elife, 2023 - elifesciences.org
Mitochondrial biogenesis requires the import of> 1,000 mitochondrial preproteins from the
cytosol. Most studies on mitochondrial protein import are focused on the core import …