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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.
TLDR
An NH2-terminal fragment of mutant huntingtin was localized to neuronal intranuclear inclusions and dystrophic neurites in the HD cortex and striatum, and polyglutamine length influenced the extent of huntingtin accumulation in these structures. Expand
Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes
TLDR
A set of guidelines for the selection and interpretation of the methods that can be used by investigators who are attempting to examine macroautophagy and related processes, as well as by reviewers who need to provide realistic and reasonable critiques of papers that investigate these processes are presented. Expand
Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation
TLDR
It is observed that mice transgenic for exon 1 of the human HD gene carrying CAG115 to (CAG)156 repeat expansions develop pronounced neuronal intranuclear inclusions, containing the proteins huntingtin and ubiquitin, prior to developing a neurological phenotype. Expand
Huntington Disease
TLDR
Individuals with the adult-onset form of Huntington disease usually live about 15 to 20 years after signs and symptoms begin, and many people with Huntington disease develop involuntary jerking or twitching movements known as chorea. Expand
Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
TLDR
Immunohistochemistry in human and rat brain revealed widespread cytoplasmic labeling of huntingtin within neurons, rather than the more selective pattern of axon terminal labeling characteristic of many vesicle-associated proteins. Expand
Altered parvalbumin-positive neuron distribution in basal ganglia of individuals with Tourette syndrome.
TLDR
The imbalance in striatal and GPi inhibitory neuron distribution suggests that the functional dynamics of cortico-striato-thalamic circuitry are fundamentally altered in severe, persistent TS. Expand
Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis
TLDR
Results support the idea that sequential proteolysis by caspase 3 and calpain may regulate huntingtin function at membranes and produce N-terminal mutant fragments that aggregate and cause cellular dysfunction in HD. Expand
Huntingtin Is Present in the Nucleus, Interacts with the Transcriptional Corepressor C-terminal Binding Protein, and Represses Transcription*
TLDR
It is speculated that wild-type huntingtin may function in the nucleus in the assembly of nuclear matrix-bound protein complexes involved with transcriptional repression and RNA processing and alter nuclear functions by disrupting protein complexes and inappropriately repressing transcription in HD. Expand
High-resolution proteomic and lipidomic analysis of exosomes and microvesicles from different cell sources
TLDR
High-resolution lipidomic and proteomic analyses of exosomes and MVs derived by differential ultracentrifugation from 3 different cell types: U87 glioblastoma cells, Huh7 hepatocellular carcinoma cells and human bone marrow-derived mesenchymal stem cells are reported. Expand
Early and Progressive Accumulation of Reactive Microglia in the Huntington Disease Brain
TLDR
The early and proximate association of activated microglia with degenerating neurons in the HD brain implicates a role for activatedmicroglia in HD pathogenesis. Expand
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