Half a century of amyloids: past, present and future

PC Ke, R Zhou, LC Serpell, R Riek… - Chemical Society …, 2020 - pubs.rsc.org
Amyloid diseases are global epidemics with profound health, social and economic
implications and yet remain without a cure. This dire situation calls for research into the …

Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities

SJ Tabrizi, MD Flower, CA Ross, EJ Wild - Nature Reviews Neurology, 2020 - nature.com
Huntington disease (HD) is a neurodegenerative disease caused by CAG repeat expansion
in the huntingtin gene (HTT) and involves a complex web of pathogenic mechanisms …

Chronic glutamate toxicity in neurodegenerative diseases—what is the evidence?

J Lewerenz, P Maher - Frontiers in neuroscience, 2015 - frontiersin.org
Together with aspartate, glutamate is the major excitatory neurotransmitter in the brain.
Glutamate binds and activates both ligand-gated ion channels (ionotropic glutamate …

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 …

Antisense oligonucleotide therapy: from design to the Huntington disease clinic

ME Rook, AL Southwell - BioDrugs, 2022 - Springer
Huntington disease (HD) is a fatal progressive neurodegenerative disorder caused by an
inherited mutation in the huntingtin (HTT) gene, which encodes mutant HTT protein. Though …

Molecular mechanisms and potential therapeutical targets in Huntington's disease

C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …

Trinucleotide repeat disorders

HT Orr, HY Zoghbi - Annu. Rev. Neurosci., 2007 - annualreviews.org
The discovery that expansion of unstable repeats can cause a variety of neurological
disorders has changed the landscape of disease-oriented research for several forms of …

Choosing an animal model for the study of Huntington's disease

MA Pouladi, AJ Morton, MR Hayden - Nature Reviews Neuroscience, 2013 - nature.com
Since the identification of the causative gene in Huntington's disease (HD), a number of
animal models of this disorder have been developed. A frequently asked question is: which …

The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology

CJ Guerriero, JL Brodsky - Physiological reviews, 2012 - journals.physiology.org
Protein folding is a complex, error-prone process that often results in an irreparable protein
by-product. These by-products can be recognized by cellular quality control machineries …

Soluble oligomers of PolyQ-expanded huntingtin target a multiplicity of key cellular factors

YE Kim, F Hosp, F Frottin, H Ge, M Mann… - Molecular cell, 2016 - cell.com
Huntington's disease is one of several neurodegenerative disorders characterized by the
aggregation of polyglutamine (polyQ)-expanded mutant protein. How polyQ aggregation …