Extracellular protein homeostasis in neurodegenerative diseases

MR Wilson, S Satapathy, M Vendruscolo - Nature Reviews Neurology, 2023 - nature.com
The protein homeostasis (proteostasis) system encompasses the cellular processes that
regulate protein synthesis, folding, concentration, trafficking and degradation. In the case of …

Advances in nuclear proteostasis of metazoans

J Buggiani, T Meinnel, C Giglione, F Frottin - Biochimie, 2024 - Elsevier
The proteostasis network and associated protein quality control (PQC) mechanisms ensure
proteome functionality and are essential for cell survival. A distinctive feature of eukaryotic …

A comprehensive enumeration of the human proteostasis network. 2. Components of the autophagy-lysosome pathway

S Elsasser, LP Elia, RI Morimoto, ET Powers… - …, 2023 - pmc.ncbi.nlm.nih.gov
The condition of having a healthy, functional proteome is known as protein homeostasis, or
proteostasis. Establishing and maintaining proteostasis is the province of the proteostasis …

[HTML][HTML] Uncovering the role of natural and synthetic small molecules in counteracting the burden of α-synuclein aggregates and related toxicity in different models of …

S Mohammed, I Russo, I Ramazzina - International Journal of Molecular …, 2023 - mdpi.com
A proteostasis network represents a sophisticated cellular system that controls the whole
process which leads to properly folded functional proteins. The imbalance of proteostasis …

A Year at the Forefront of Proteostasis and Aging

MA Thompson, EA De-Souza - Biology Open, 2023 - journals.biologists.com
During aging, animals experience a decline in proteostasis activity, including loss of stress-
response activation, culminating in the accumulation of misfolded proteins and toxic …

Map** stress-responsive signaling pathways induced by mitochondrial proteostasis perturbations

N Madrazo, Z Khattar, ET Powers… - Molecular Biology of …, 2024 - molbiolcell.org
Imbalances in mitochondrial proteostasis are associated with pathologic mitochondrial
dysfunction implicated in etiologically diverse diseases. This has led to considerable interest …

Mitochondrial dysfunction rapidly modulates the abundance and thermal stability of cellular proteins

C Groh, P Haberkant, F Stein, S Filbeck… - Life science …, 2023 - life-science-alliance.org
Cellular functionality relies on a well-balanced, but highly dynamic proteome. Dysfunction of
mitochondrial protein import leads to the cytosolic accumulation of mitochondrial precursor …

Development of quantitative high-throughput screening assays to identify, validate, and optimize small-molecule stabilizers of misfolded β-glucocerebrosidase with …

D Williams, LM Glasstetter, TT Jong, A Kapoor, S Zhu… - …, 2024 - pmc.ncbi.nlm.nih.gov
Glucocerebrosidase (GCase) is implicated in both a rare, monogenic disorder (Gaucher
disease, GD) and a common, multifactorial condition (Parkinson's disease); hence, it is an …

Single-cell expression predicts neuron-specific protein homeostasis networks

S Pechmann - Open Biology, 2024 - royalsocietypublishing.org
The protein homeostasis network keeps proteins in their correct shapes and avoids
unwanted aggregation. In turn, the accumulation of aberrantly misfolded proteins has been …

TTC17 is an endoplasmic reticulum resident TPR-containing adaptor protein

NP Canniff, JB Graham, KP Guay, DA Lubicki… - Journal of Biological …, 2023 - jbc.org
Protein folding, quality control, maturation, and trafficking are essential processes for proper
cellular homeostasis. Around one-third of the human proteome is targeted to the …