Amyloid formation as a protein phase transition

TCT Michaels, D Qian, A Šarić, M Vendruscolo… - Nature Reviews …, 2023 - nature.com
The formation of amyloid fibrils is a general class of protein self-assembly behaviour, which
is associated with both functional biology and the development of a number of disorders …

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 …

Regulation of heat shock transcription factors and their roles in physiology and disease

R Gomez-Pastor, ET Burchfiel, DJ Thiele - Nature reviews Molecular …, 2018 - nature.com
The heat shock transcription factors (HSFs) were discovered over 30 years ago as direct
transcriptional activators of genes regulated by thermal stress, encoding heat shock …

Protein quality control by molecular chaperones in neurodegeneration

A Ciechanover, YT Kwon - Frontiers in neuroscience, 2017 - frontiersin.org
Protein homeostasis (proteostasis) requires the timely degradation of misfolded proteins and
their aggregates by protein quality control (PQC), of which molecular chaperones are an …

Huntington disease

GP Bates, R Dorsey, JF Gusella, MR Hayden… - Nature reviews Disease …, 2015 - nature.com
Huntington disease is devastating to patients and their families—with autosomal dominant
inheritance, onset typically in the prime of adult life, progressive course, and a combination …

Functional modules of the proteostasis network

GG Jayaraj, MS Hipp, FU Hartl - Cold Spring Harbor …, 2020 - cshperspectives.cshlp.org
Cells invest in an extensive network of factors to maintain protein homeostasis (proteostasis)
and prevent the accumulation of potentially toxic protein aggregates. This proteostasis …

Huntington's disease: underlying molecular mechanisms and emerging concepts

J Labbadia, RI Morimoto - Trends in biochemical sciences, 2013 - cell.com
Huntington's disease (HD) is a progressive neurodegenerative disorder for which no
disease modifying treatments exist. Many molecular changes and cellular consequences …

Clinical features of Huntington's disease

R Ghosh, SJ Tabrizi - Polyglutamine disorders, 2018 - Springer
Huntington's disease (HD) is the most common monogenic neurodegenerative disease and
the commonest genetic dementia in the developed world. With autosomal dominant …

Shaping proteostasis at the cellular, tissue, and organismal level

AJ Sala, LC Bott, RI Morimoto - Journal of Cell Biology, 2017 - rupress.org
The proteostasis network (PN) regulates protein synthesis, folding, transport, and
degradation to maintain proteome integrity and limit the accumulation of protein aggregates …

Molecular chaperones: a double-edged sword in neurodegenerative diseases

J Tittelmeier, E Nachman… - Frontiers in aging …, 2020 - frontiersin.org
Aberrant accumulation of misfolded proteins into amyloid deposits is a hallmark in many age-
related neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's …