Mechanisms of autophagy–lysosome dysfunction in neurodegenerative diseases

RA Nixon, DC Rubinsztein - Nature Reviews Molecular Cell Biology, 2024 - nature.com
Autophagy is a lysosome-based degradative process used to recycle obsolete cellular
constituents and eliminate damaged organelles and aggregate-prone proteins. Their …

Functional amyloids

D Otzen, R Riek - Cold Spring Harbor perspectives in …, 2019 - cshperspectives.cshlp.org
When protein/peptides aggregate, they usually form the amyloid state consisting of cross β-
sheet structure built by repetitively stacked β-strands forming long fibrils. Amyloids are …

Orchestration of selective autophagy by cargo receptors

E Adriaenssens, L Ferrari, S Martens - Current Biology, 2022 - cell.com
Cellular homeostasis requires the swift and specific removal of damaged material. Selective
autophagy represents a major pathway for the degradation of such cargo material. This is …

The Hsp90 molecular chaperone governs client proteins by targeting intrinsically disordered regions

JA Kolhe, NL Babu, BC Freeman - Molecular cell, 2023 - cell.com
Molecular chaperones govern proteome health to support cell homeostasis. An essential
eukaryotic component of the chaperone system is Hsp90. Using a chemical-biology …

Molecular mechanisms of heat shock factor 1 regulation

SW Kmiecik, MP Mayer - Trends in biochemical sciences, 2022 - cell.com
To thrive and to fulfill their functions, cells need to maintain proteome homeostasis even in
the face of adverse environmental conditions or radical restructuring of the proteome during …

Mechanisms tailoring the expression of heat shock proteins to proteostasis challenges

LRA Boopathy, S Jacob-Tomas, C Alecki… - Journal of Biological …, 2022 - ASBMB
All cells possess an internal stress response to cope with environmental and
pathophysiological challenges. Upon stress, cells reprogram their molecular functions to …

Adapting the proteostasis capacity to sustain brain healthspan

C Hetz - Cell, 2021 - cell.com
Sustaining neuronal proteostasis during the course of our life is a central aspect required for
brain function. The dynamic nature of synaptic composition and abundance is a requisite to …

The self-association equilibrium of DNAJA2 regulates its interaction with unfolded substrate proteins and with Hsc70

L Velasco-Carneros, J Cuéllar, L Dublang… - Nature …, 2023 - nature.com
J-domain proteins tune the specificity of Hsp70s, engaging them in precise functions.
Despite their essential role, the structure and function of many J-domain proteins remain …

Alternative systems for misfolded protein clearance: life beyond the proteasome

HE Johnston, RS Samant - The FEBS journal, 2021 - Wiley Online Library
Protein misfolding is a major driver of ageing‐associated frailty and disease pathology.
Although all cells possess multiple, well‐characterised protein quality control systems to …

Loss of TDP‐43 oligomerization or RNA binding elicits distinct aggregation patterns

M Pérez‐Berlanga, VI Wiersma, A Zbinden… - The EMBO …, 2023 - embopress.org
Aggregation of the RNA‐binding protein TAR DNA‐binding protein 43 (TDP‐43) is the key
neuropathological feature of neurodegenerative diseases, including amyotrophic lateral …