The proteostasis network and its decline in ageing
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 …
neurodegenerative disorders such as Alzheimer disease and Parkinson disease. A hallmark …
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 …
synthesis, folding, conformational maintenance, and degradation. A complex and adaptive …
The nucleolus functions as a phase-separated protein quality control compartment
INTRODUCTION Cells have evolved quality control mechanisms that operate under normal
growth conditions and during stress to maintain protein homeostasis (proteostasis) and …
growth conditions and during stress to maintain protein homeostasis (proteostasis) and …
In vivo aspects of protein folding and quality control
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 …
must fold into unique three-dimensional structures to fulfill their biological functions. Protein …
[HTML][HTML] In situ structure of neuronal C9orf72 poly-GA aggregates reveals proteasome recruitment
Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of
many neurodegenerative diseases. Here, we address the elusive link between these …
many neurodegenerative diseases. Here, we address the elusive link between these …
The amyloid state and its association with protein misfolding diseases
TPJ Knowles, M Vendruscolo… - Nature reviews Molecular …, 2014 - nature.com
The phenomenon of protein aggregation and amyloid formation has become the subject of
rapidly increasing research activities across a wide range of scientific disciplines. Such …
rapidly increasing research activities across a wide range of scientific disciplines. Such …
An aberrant phase transition of stress granules triggered by misfolded protein and prevented by chaperone function
Stress granules (SG) are membrane‐less compartments involved in regulating mRNAs
during stress. Aberrant forms of SGs have been implicated in age‐related diseases, such as …
during stress. Aberrant forms of SGs have been implicated in age‐related diseases, such as …
Protein misfolding diseases
FU Hartl - Annual review of biochemistry, 2017 - annualreviews.org
The majority of protein molecules must fold into defined three-dimensional structures to
acquire functional activity. However, protein chains can adopt a multitude of conformational …
acquire functional activity. However, protein chains can adopt a multitude of conformational …
The biology of proteostasis in aging and disease
J Labbadia, RI Morimoto - Annual review of biochemistry, 2015 - annualreviews.org
Loss of protein homeostasis (proteostasis) is a common feature of aging and disease that is
characterized by the appearance of nonnative protein aggregates in various tissues. Protein …
characterized by the appearance of nonnative protein aggregates in various tissues. Protein …
A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease
Chaperones are central to the proteostasis network (PN) and safeguard the proteome from
misfolding, aggregation, and proteotoxicity. We categorized the human chaperome of 332 …
misfolding, aggregation, and proteotoxicity. We categorized the human chaperome of 332 …