ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin. [PDF]
PLoS ONE, 2014 A hallmark of Huntington's disease is the pronounced sensitivity of striatal neurons to polyglutamine-expanded huntingtin expression. Here we show that cultured striatal cells and murine brain striatum have remarkably low levels of phosphorylation of ...
Julia Leitman +6 more
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The Ubiquitin-Proteasome Pathway in Huntington's Disease [PDF]
The Scientific World Journal, 2008 The accumulation of mutant protein is a common feature of neurodegenerative disease. In Huntington's disease, a polyglutamine expansion in the huntingtin protein triggers neuronal toxicity.
Siddhartha Mitra, Steven Finkbeiner
doaj +3 more sources
Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons [PDF]
Molecular Neurodegeneration, 2011 Background Huntington's disease is caused by aggregation of mutant huntingtin (mHtt) protein containing more than a 36 polyQ repeat. Upregulation of macroautophagy was suggested as a neuroprotective strategy to degrade mutant huntingtin.
Ouyang Xiaosen +3 more
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High-content chemical and RNAi screens for suppressors of neurotoxicity in a Huntington's disease model. [PDF]
PLoS ONE, 2011 To identify Huntington's Disease therapeutics, we conducted high-content small molecule and RNAi suppressor screens using a Drosophila primary neural culture Huntingtin model.
Joost Schulte +4 more
doaj +5 more sources
Differential effects of overexpression of mutant huntingtin and TDP-43 in agouti-related protein neurons in the arcuate nucleus of the hypothalamus in mice. [PDF]
Acta Neuropathol CommunOraha J +5 more
europepmc +2 more sources
Frontiers in Physiology, 2023 Introduction: Huntington disease is an autosomal dominant neurodegenerative disorder which is caused by a CAG repeat expansion in the HTT gene that codes for an elongated polyglutamine tract in the huntingtin (HTT) protein.
Fanny L. Lemarié +4 more
doaj +1 more source
Huntingtin is critical both pre- and postsynaptically for long-term learning-related synaptic plasticity in Aplysia. [PDF]
PLoS ONE, 2014 Patients with Huntington's disease exhibit memory and cognitive deficits many years before manifesting motor disturbances. Similarly, several studies have shown that deficits in long-term synaptic plasticity, a cellular basis of memory formation and ...
Yun-Beom Choi +5 more
doaj +1 more source
Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain [PDF]
Nature Communications, 2015 Ellen J Spartz, Weizhe Hong, Liqun Luo
exaly +2 more sources
Frontiers in Molecular Biosciences, 2023 Polyglutamine expansion at the N-terminus of the huntingtin protein exon 1 (Htt-ex1) is closely associated with a number of neurodegenerative diseases, which result from the aggregation of the increased polyQ repeat.
Mohammed Khaled +3 more
doaj +1 more source
Effect of post-mortem delay on N-terminal huntingtin protein fragments in human control and Huntington disease brain lysates. [PDF]
PLoS ONE, 2017 Huntington disease is associated with elongation of a CAG repeat in the HTT gene that results in a mutant huntingtin protein. Several studies have implicated N-terminal huntingtin protein fragments in Huntington disease pathogenesis.
Menno H Schut +9 more
doaj +1 more source