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 …

Mitochondrial complexome and import network

F den Brave, U Schulte, B Fakler, N Pfanner… - Trends in Cell Biology, 2024 - cell.com
Mitochondria perform crucial functions in cellular metabolism, protein and lipid biogenesis,
quality control, and signaling. The systematic analysis of protein complexes and interaction …

Mitochondrial complexome reveals quality-control pathways of protein import

U Schulte, F den Brave, A Haupt, A Gupta, J Song… - Nature, 2023 - nature.com
Mitochondria have crucial roles in cellular energetics, metabolism, signalling and quality
control,,–. They contain around 1,000 different proteins that often assemble into complexes …

[HTML][HTML] Mitochondrial entry gate as regulatory hub

F den Brave, N Pfanner, T Becker - … et Biophysica Acta (BBA)-Molecular Cell …, 2024 - Elsevier
Mitochondria import 1000–1300 different precursor proteins from the cytosol. The main
mitochondrial entry gate is formed by the translocase of the outer membrane (TOM …

The role of the proteasome in mitochondrial protein quality control

S Rödl, JM Herrmann - IUBMB life, 2023 - Wiley Online Library
The abundance of each cellular protein is dynamically adjusted to the prevailing metabolic
and stress conditions by modulation of their synthesis and degradation rates. The …

Nuclear ubiquitin-proteasome pathways in proteostasis maintenance

D Franić, K Zubčić, M Boban - Biomolecules, 2021 - mdpi.com
Protein homeostasis, or proteostasis, is crucial for the functioning of a cell, as proteins that
are mislocalized, present in excessive amounts, or aberrant due to misfolding or other type …

Nuclear import carrier Hikeshi cooperates with HSP70 to promote murine oligodendrocyte differentiation and CNS myelination

L Li, D Li, D Sun, X Zhang, W Lei, M Wu, Q Huang… - Developmental Cell, 2023 - cell.com
Dysregulation of factors in nucleocytoplasmic transport is closely linked to neural
developmental diseases. Mutation in Hikeshi, encoding a nonconventional nuclear import …

Ubiquitin ligase redundancy and nuclear-cytoplasmic localization in yeast protein quality control

CA Breckel, M Hochstrasser - Biomolecules, 2021 - mdpi.com
The diverse functions of proteins depend on their proper three-dimensional folding and
assembly. Misfolded cellular proteins can potentially harm cells by forming aggregates in …

Role of J-domain proteins in yeast physiology and protein quality control

C Ruger-Herreros, L Svoboda, A Mogk… - Journal of Molecular …, 2024 - Elsevier
The Hsp70 chaperone system is a central component of cellular protein quality control
(PQC) by acting in a multitude of protein folding processes ranging from the folding of newly …

Molecular mechanisms of amyotrophic lateral sclerosis as broad therapeutic targets for gene therapy applications utilizing adeno‐associated viral vectors

J Merjane, R Chung, R Patani… - Medicinal Research …, 2023 - Wiley Online Library
Despite the devastating clinical outcome of the neurodegenerative disease, amyotrophic
lateral sclerosis (ALS), its etiology remains mysterious. Approximately 90% of ALS is …