Half a century of amyloids: past, present and future
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 …
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
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 …
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 …
Glutamate binds and activates both ligand-gated ion channels (ionotropic glutamate …
A liquid to solid phase transition underlying pathological huntingtin exon1 aggregation
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 …
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 …
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 …
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …
Trinucleotide repeat disorders
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 …
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 …
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 …
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
Huntington's disease is one of several neurodegenerative disorders characterized by the
aggregation of polyglutamine (polyQ)-expanded mutant protein. How polyQ aggregation …
aggregation of polyglutamine (polyQ)-expanded mutant protein. How polyQ aggregation …