Mitochondrial quality control in health and in Parkinson's disease

MA Eldeeb, RA Thomas, MA Ragheb… - Physiological …, 2022 - journals.physiology.org
As a central hub for cellular metabolism and intracellular signaling, the mitochondrion is a
pivotal organelle, dysfunction of which has been linked to several human diseases including …

Quality control of the mitochondrial proteome

J Song, JM Herrmann, T Becker - Nature Reviews Molecular Cell …, 2021 - nature.com
Abstract Mitochondria contain about 1,000–1,500 proteins that fulfil multiple functions.
Mitochondrial proteins originate from two genomes: mitochondrial and nuclear. Hence …

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 machineries for protein import and assembly

N Wiedemann, N Pfanner - Annual review of biochemistry, 2017 - annualreviews.org
Mitochondria are essential organelles with numerous functions in cellular metabolism and
homeostasis. Most of the> 1,000 different mitochondrial proteins are synthesized as …

Tom70 enhances mitochondrial preprotein import efficiency by binding to internal targeting sequences

S Backes, S Hess, F Boos, MW Woellhaf… - Journal of Cell …, 2018 - rupress.org
The biogenesis of mitochondria depends on the import of hundreds of preproteins. N-
terminal matrix-targeting signals (MTSs) direct preproteins to the surface receptors Tom20 …

An ER surface retrieval pathway safeguards the import of mitochondrial membrane proteins in yeast

KG Hansen, N Aviram, J Laborenz, C Bibi, M Meyer… - Science, 2018 - science.org
The majority of organellar proteins are translated on cytosolic ribosomes and must be sorted
correctly to function. Targeting routes have been identified for organelles such as …

A new mitofusin topology places the redox-regulated C terminus in the mitochondrial intermembrane space

S Mattie, J Riemer, JG Wideman, HM McBride - Journal of Cell Biology, 2018 - rupress.org
Mitochondrial fusion occurs in many eukaryotes, including animals, plants, and fungi. It is
essential for cellular homeostasis, and yet the underlying mechanisms remain elusive …

Cytosolic events in the biogenesis of mitochondrial proteins

YS Bykov, D Rapaport, JM Herrmann… - Trends in biochemical …, 2020 - cell.com
While targeting of proteins synthesized in the cytosol to any organelle is complex,
mitochondria present the most challenging of destinations. First, import of nuclear-encoded …

The mitochondrial import complex MIM functions as main translocase for α-helical outer membrane proteins

KN Doan, A Grevel, CU Mårtensson, L Ellenrieder… - Cell reports, 2020 - cell.com
The mitochondrial outer membrane contains integral proteins with α-helical membrane
anchors or a transmembrane β-barrel. The translocase of the outer membrane (TOM) …

The ER-SURF pathway uses ER-mitochondria contact sites for protein targeting to mitochondria

C Koch, S Lenhard, M Räschle… - EMBO …, 2024 - embopress.org
Most mitochondrial proteins are synthesized on cytosolic ribosomes and imported into
mitochondria in a post-translational reaction. Mitochondrial precursor proteins which use the …