Physicochemical properties of cells and their effects on intrinsically disordered proteins (IDPs)

FX Theillet, A Binolfi, T Frembgen-Kesner… - Chemical …, 2014 - ACS Publications
It has long been axiomatic that a protein's structure determines its function. Intrinsically
disordered proteins (IDPs) and disordered protein regions (IDRs) defy this structure …

Protein aggregates in Huntington's disease

M Arrasate, S Finkbeiner - Experimental neurology, 2012 - Elsevier
Huntington's disease (HD) is an incurable neurodegenerative disease characterized by
abnormal motor movements, personality changes, and early death. HD is caused by a …

A liquid to solid phase transition underlying pathological huntingtin exon1 aggregation

TR Peskett, F Rau, J O'Driscoll, R Patani, AR Lowe… - Molecular cell, 2018 - cell.com
Huntington's disease is caused by an abnormally long polyglutamine tract in the huntingtin
protein. This leads to the generation and deposition of N-terminal exon1 fragments of the …

Aneuploidy causes proteotoxic stress in yeast

AB Oromendia, SE Dodgson, A Amon - Genes & development, 2012 - genesdev.cshlp.org
Gains or losses of entire chromosomes lead to aneuploidy, a condition tolerated poorly in all
eukaryotes analyzed to date. How aneuploidy affects organismal and cellular physiology is …

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

BS Johnson, JM McCaffery… - Proceedings of the …, 2008 - National Acad Sciences
Protein misfolding is intimately associated with devastating human neurodegenerative
diseases, including Alzheimer's, Huntington's, and Parkinson's. Although disparate in their …

Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism

AK Thakur, M Jayaraman, R Mishra, M Thakur… - Nature structural & …, 2009 - nature.com
Simple polyglutamine (polyQ) peptides aggregate in vitro via a nucleated growth pathway
directly yielding amyloid-like aggregates. We show here that the 17-amino-acid flanking …

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

RS Atwal, J Xia, D Pinchev, J Taylor… - Human molecular …, 2007 - academic.oup.com
Huntington's disease is caused by an expanded polyglutamine tract in huntingtin protein,
leading to accumulation of huntingtin in the nuclei of striatal neurons. The 18 amino-acid …

Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice

X Gu, ER Greiner, R Mishra, R Kodali, A Osmand… - Neuron, 2009 - cell.com
The N-terminal 17 amino acids of huntingtin (NT17) can be phosphorylated on serines 13
and 16; however, the significance of these modifications in Huntington's disease …

Unmasking the roles of N-and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation

SL Crick, KM Ruff, K Garai… - Proceedings of the …, 2013 - National Acad Sciences
Huntington disease is caused by mutational expansion of the CAG trinucleotide within exon
1 of the huntingtin (Htt) gene. Exon 1 spanning N-terminal fragments (NTFs) of the Htt …

Modelling neurodegeneration in Saccharomyces cerevisiae: why cook with baker's yeast?

V Khurana, S Lindquist - Nature Reviews Neuroscience, 2010 - nature.com
In ageing populations, neurodegenerative diseases increase in prevalence, exacting an
enormous toll on individuals and their communities. Multiple complementary experimental …