Protein misfolding and ER stress in Huntington's disease

T Shacham, N Sharma… - Frontiers in molecular …, 2019 - frontiersin.org
Increasing evidence in recent years indicates that protein misfolding and aggregation,
leading to ER stress, are central factors of pathogenicity in neurodegenerative diseases …

Endoplasmic reticulum stress: The cause and solution to Huntington's disease?

Y Jiang, SR Chadwick, P Lajoie - Brain research, 2016 - Elsevier
Accumulation of misfolded proteins is a hallmark of many human diseases, including several
incurable neurological disorders, such as Huntington's disease (HD). In HD, expansion of a …

Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's disease

R Vidal, B Caballero, A Couve… - Current molecular …, 2011 - ingentaconnect.com
A variety of neurological diseases including Huntington's disease (HD), Alzheimer's disease
and Parkinson's disease share common neuropathology, primarily featuring the presence of …

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

J Leitman, F Ulrich Hartl, GZ Lederkremer - Nature communications, 2013 - nature.com
In Huntington's disease, as in other neurodegenerative diseases, it was initially thought that
insoluble protein aggregates are the toxic species. However, growing evidence implicates …

Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins

S Reijonen, N Putkonen, A Nørremølle… - Experimental cell …, 2008 - Elsevier
Accumulation of abnormal proteins occurs in many neurodegenerative diseases including
Huntington's disease (HD). However, the precise role of protein aggregation in neuronal cell …

Neurodegenerative processes in Huntington's disease

D Bano, F Zanetti, Y Mende, P Nicotera - Cell death & disease, 2011 - nature.com
Huntington's disease (HD) is a complex and severe disorder characterized by the gradual
and the progressive loss of neurons, predominantly in the striatum, which leads to the typical …

An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity

G Cisbani, F Cicchetti - Cell death & disease, 2012 - nature.com
Huntington's disease (HD) is a devastating neurodegenerative disorder whose main
hallmark is brain atrophy. However, several peripheral organs are considerably affected and …

In vitro and in vivo aggregation of a fragment of huntingtin protein directly causes free radical production

S Hands, MU Sajjad, MJ Newton… - Journal of Biological …, 2011 - ASBMB
Neurodegenerative diseases are characterized by intra-and/or extracellular protein
aggregation and oxidative stress. Intense attention has been paid to whether protein …

Phosphorylation of huntingtin at residue T3 is decreased in Huntington's disease and modulates mutant huntingtin protein conformation

C Cariulo, L Azzollini, M Verani… - Proceedings of the …, 2017 - National Acad Sciences
Posttranslational modifications can have profound effects on the biological and biophysical
properties of proteins associated with misfolding and aggregation. However, their detection …

The pathobiology of perturbed mutant huntingtin protein–protein interactions in Huntington's disease

EE Wanker, A Ast, F Schindler, P Trepte… - Journal of …, 2019 - Wiley Online Library
Mutations are at the root of many human diseases. Still, we largely do not exactly
understand how they trigger pathogenesis. One, more recent, hypothesis has been that they …