[PDF][PDF] 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 …

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 …

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 …

Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice

Nature neuroscience, 2017 - nature.com
Neural cultures derived from Huntington's disease (HD) patient-derived induced pluripotent
stem cells were used for'omics' analyses to identify mechanisms underlying …

Huntingtin–protein interactions and the pathogenesis of Huntington's disease

SH Li, XJ Li - TRENDS in Genetics, 2004 - cell.com
At least nine inherited neurodegenerative diseases share a polyglutamine expansion in
their respective disease proteins. These diseases show distinct neuropathological changes …

Mechanisms of neurodegeneration in Huntington's disease

JM Gil, AC Rego - European Journal of Neuroscience, 2008 - Wiley Online Library
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 …

Proteins containing expanded polyglutamine tracts and neurodegenerative disease

A Adegbuyiro, F Sedighi, AW Pilkington IV… - Biochemistry, 2017 - ACS Publications
Several hereditary neurological and neuromuscular diseases are caused by an abnormal
expansion of trinucleotide repeats. To date, there have been 10 of these trinucleotide repeat …

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 …

[HTML][HTML] DNA damage repair in Huntington's disease and other neurodegenerative diseases

T Maiuri, CE Suart, CLK Hung, KJ Graham… - …, 2019 - Elsevier
Recent genome-wide association studies of Huntington's disease (HD) primarily highlighted
genes involved in DNA damage repair mechanisms as modifiers of age at onset and …

[HTML][HTML] Physical exercise-induced adult neurogenesis: a good strategy to prevent cognitive decline in neurodegenerative diseases?

S Yau, J Gil-Mohapel, BR Christie, K So - BioMed research …, 2014 - hindawi.com
Cumulative evidence has indicated that there is an important role for adult hippocampal
neurogenesis in cognitive function. With the increasing prevalence of cognitive decline …