The proteostasis network and its decline in ageing

MS Hipp, P Kasturi, FU Hartl - Nature reviews Molecular cell biology, 2019 - nature.com
Ageing is a major risk factor for the development of many diseases, prominently including
neurodegenerative disorders such as Alzheimer disease and Parkinson disease. A hallmark …

Half a century of amyloids: past, present and future

PC Ke, R Zhou, LC Serpell, R Riek… - Chemical Society …, 2020 - pubs.rsc.org
Amyloid diseases are global epidemics with profound health, social and economic
implications and yet remain without a cure. This dire situation calls for research into the …

A new era for understanding amyloid structures and disease

MG Iadanza, MP Jackson, EW Hewitt… - … reviews Molecular cell …, 2018 - nature.com
The aggregation of proteins into amyloid fibrils and their deposition into plaques and
intracellular inclusions is the hallmark of amyloid disease. The accumulation and deposition …

Molecular mechanisms underlying nucleotide repeat expansion disorders

I Malik, CP Kelley, ET Wang, PK Todd - Nature reviews Molecular cell …, 2021 - nature.com
The human genome contains over one million short tandem repeats. Expansion of a subset
of these repeat tracts underlies over fifty human disorders, including common genetic …

Capillary forces generated by biomolecular condensates

B Gouveia, Y Kim, JW Shaevitz, S Petry, HA Stone… - Nature, 2022 - nature.com
Liquid–liquid phase separation and related phase transitions have emerged as generic
mechanisms in living cells for the formation of membraneless compartments or biomolecular …

Mechanisms and pathology of protein misfolding and aggregation

N Louros, J Schymkowitz, F Rousseau - Nature Reviews Molecular Cell …, 2023 - nature.com
Despite advances in machine learning-based protein structure prediction, we are still far
from fully understanding how proteins fold into their native conformation. The conventional …

Pathways of cellular proteostasis in aging and disease

CL Klaips, GG Jayaraj, FU Hartl - Journal of Cell Biology, 2018 - rupress.org
Ensuring cellular protein homeostasis, or proteostasis, requires precise control of protein
synthesis, folding, conformational maintenance, and degradation. A complex and adaptive …

Cryo-electron tomography on focused ion beam lamellae transforms structural cell biology

C Berger, N Premaraj, RBG Ravelli, K Knoops… - Nature …, 2023 - nature.com
Cryogenic electron microscopy and data processing enable the determination of structures
of isolated macromolecules to near-atomic resolution. However, these data do not provide …

[HTML][HTML] In situ structure of neuronal C9orf72 poly-GA aggregates reveals proteasome recruitment

Q Guo, C Lehmer, A Martínez-Sánchez, T Rudack… - Cell, 2018 - cell.com
Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of
many neurodegenerative diseases. Here, we address the elusive link between these …

Extracellular protein components of amyloid plaques and their roles in Alzheimer's disease pathology

MM Rahman, C Lendel - Molecular Neurodegeneration, 2021 - Springer
Alzheimer's disease (AD) is pathologically defined by the presence of fibrillar amyloid β (Aβ)
peptide in extracellular senile plaques and tau filaments in intracellular neurofibrillary …