The biology of huntingtin
F Saudou, S Humbert - Neuron, 2016 - cell.com
Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
[HTML][HTML] Autophagy and apoptosis dysfunction in neurodegenerative disorders
Autophagy and apoptosis are basic physiologic processes contributing to the maintenance
of cellular homeostasis. Autophagy encompasses pathways that target long-lived cytosolic …
of cellular homeostasis. Autophagy encompasses pathways that target long-lived cytosolic …
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 …
Long non-coding RNAs in Huntington's disease neurodegeneration
R Johnson - Neurobiology of disease, 2012 - Elsevier
Neurodegeneration in the brains of Huntington's disease patients is accompanied by
widespread changes in gene regulatory networks. Recent studies have found that these …
widespread changes in gene regulatory networks. Recent studies have found that these …
Normal huntingtin function: an alternative approach to Huntington's disease
E Cattaneo, C Zuccato, M Tartari - Nature Reviews Neuroscience, 2005 - nature.com
Several neurological diseases are characterized by the altered activity of one or a few
ubiquitously expressed cell proteins, but it is not known how these normal proteins turn into …
ubiquitously expressed cell proteins, but it is not known how these normal proteins turn into …
Metabolic dysfunction in Alzheimer's disease and related neurodegenerative disorders
Alzheimer's disease and other related neurodegenerative diseases are highly debilitating
disorders that affect millions of people worldwide. Efforts towards developing effective …
disorders that affect millions of people worldwide. Efforts towards developing effective …
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin
RK Graham, Y Deng, EJ Slow, B Haigh, N Bissada… - Cell, 2006 - cell.com
Cleavage of huntingtin (htt) has been characterized in vitro, and accumulation of caspase
cleavage fragments represents an early pathological change in brains of Huntington's …
cleavage fragments represents an early pathological change in brains of Huntington's …
Mechanisms of neurodegeneration in Huntington's disease
Huntington's disease (HD) is caused by an expansion of cytosine–adenine–guanine (CAG)
repeats in the huntingtin gene, which leads to neuronal loss in the striatum and cortex and to …
repeats in the huntingtin gene, which leads to neuronal loss in the striatum and cortex and to …
Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases
E Trushina, CT McMurray - Neuroscience, 2007 - Elsevier
In recent years, it has become increasingly clear that mitochondrial dysfunction and
oxidative damage are major contributors to neuronal loss. Free radicals, typically generated …
oxidative damage are major contributors to neuronal loss. Free radicals, typically generated …