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 …

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 …

The nucleolus functions as a phase-separated protein quality control compartment

F Frottin, F Schueder, S Tiwary, R Gupta, R Körner… - Science, 2019 - science.org
INTRODUCTION Cells have evolved quality control mechanisms that operate under normal
growth conditions and during stress to maintain protein homeostasis (proteostasis) and …

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 …

In vivo aspects of protein folding and quality control

D Balchin, M Hayer-Hartl, FU Hartl - Science, 2016 - science.org
BACKGROUND Proteins are synthesized on ribosomes as linear chains of amino acids and
must fold into unique three-dimensional structures to fulfill their biological functions. Protein …

Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation

P Tziortzouda, L Van Den Bosch, F Hirth - Nature Reviews …, 2021 - nature.com
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

CC Chou, YI Zhang, ME Umoh, SW Vaughan… - Nature …, 2018 - nature.com
The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43)
is a common histopathological hallmark of the amyotrophic lateral sclerosis and …

Protein misfolding in neurodegenerative diseases: implications and strategies

P Sweeney, H Park, M Baumann, J Dunlop… - Translational …, 2017 - Springer
A hallmark of neurodegenerative proteinopathies is the formation of misfolded protein
aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse …

Size distributions of intracellular condensates reflect competition between coalescence and nucleation

DSW Lee, CH Choi, DW Sanders, L Beckers… - Nature Physics, 2023 - nature.com
Phase separation of biomolecules into condensates has emerged as a mechanism for
intracellular organization and affects many intracellular processes, including reaction …

Tau protein disrupts nucleocytoplasmic transport in Alzheimer's disease

B Eftekharzadeh, JG Daigle, LE Kapinos, A Coyne… - Neuron, 2018 - cell.com
Tau is the major constituent of neurofibrillary tangles in Alzheimer's disease (AD), but the
mechanism underlying tau-associated neural damage remains unclear. Here, we show that …