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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
A Human Protein-Protein Interaction Network: A Resource for Annotating the Proteome
TLDR
A large, highly connected network of interacting pairs of human proteins was identified, characterizing ANP32A and CRMP1 as modulators of Wnt signaling and two novel Axin-1 interactions were validated experimentally. Expand
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription.
TLDR
The possibility that expanded repeat htt causes aberrant transcriptional regulation through its interaction with cellular transcription factors which may result in neuronal dysfunction and cell death in HD is raised. Expand
Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo
TLDR
In this study, it is shown that the proteolytic cleavage of a GST-huntingtin fusion protein leads to the formation of insoluble high molecular weight protein aggregates only when the polyglutamine expansion is in the pathogenic range. Expand
EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers
TLDR
Redirection of amyloid fibril formation through the action of a small molecule is demonstrated, resulting in off-pathway, highly stable oligomers, suggesting a generic effect on aggregation pathways in neurodegenerative diseases. Expand
The hunt for huntingtin function: interaction partners tell many different stories.
TLDR
Research in past years has identified several htt-interacting proteins that play roles in clathrin-mediated endocytosis, apoptosis, vesicle transport, cell signalling, morphogenesis and transcriptional regulation, suggesting that htt is also involved in these processes. Expand
HIPPIE: Integrating Protein Interaction Networks with Experiment Based Quality Scores
TLDR
HIPPIE (Human Integrated Protein-Protein Interaction rEference) is generated, a human PPI dataset with a normalized scoring scheme that integrates multiple experimental PPI datasets and it is shown that these scores correlate to the quality of the experimental characterization. Expand
EGCG remodels mature α-synuclein and amyloid-β fibrils and reduces cellular toxicity
TLDR
It is shown that EGCG has the ability to convert large, mature α-synuclein and amyloid-β fibrils into smaller, amorphous protein aggregates that are nontoxic to mammalian cells. Expand
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation.
TLDR
In 293 Tet-Off cells, inclusion body formation resulted in cell toxicity and dramatic ultrastructural changes such as indentations and disruption of the nuclear envelope, which support the hypothesis that the ATP-dependent ubiquitin-proteasome system is a potential target for therapeutic interventions in glutamine repeat disorders. Expand
A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease.
TLDR
A strategy combining library and matrix yeast two-hybrid screens to generate a highly connected PPI network for Huntington's disease (HD) revealed 165 new potential interactions and facilitated the discovery of GIT1, a G protein-coupled receptor kinase-interacting protein, which enhances huntingtin aggregation by recruitment of the protein into membranous vesicles. Expand
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