Results 31 to 40 of about 19,163 (188)
Forms and Phases in Huntingtin Protein Aggregation [PDF]
Molecular Cell, 2018 Using a combination of fluorescence microscopy and electron tomography, Peskett et al. (2018), in this issue of Molecular Cell, explore the nucleation of amyloid-like filaments from liquid-like condensates of huntingtin protein exon1 with disease-related polyQ extensions.
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Nature Communications, 2021 The mechanisms underlying Huntingtin protein (Htt) aggregation are not fully understood. Here the authors perform a detailed investigation of the ultrastructural and biochemical properties of huntingtin cytoplasmic and nuclear inclusions, and reveal that
Nathan Riguet +11 more
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Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease
Neurobiology of Disease, 2007 Recent evidence suggests that mutant huntingtin protein-induced energetic perturbations contribute to neuronal dysfunction in Huntington’s disease (HD). Given the ubiquitous expression of huntingtin, other cell types with high energetic burden may be at ...
Michael J. Mihm +5 more
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DISC1 and Huntington's disease--overlapping pathways of vulnerability to neurological disorder? [PDF]
PLoS ONE, 2011 We re-annotated the interacting partners of the neuronal scaffold protein DISC1 using a knowledge-based approach that incorporated recent protein interaction data and published literature to. This revealed two highly connected networks.
Ruth Boxall +2 more
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Depletion of CBP is directly linked with cellular toxicity caused by mutant huntingtin
Neurobiology of Disease, 2006 Huntington's disease is a neurodegenerative disease caused by an expanded polyglutamine stretch within the huntingtin protein. Transfection of mutant huntingtin causes cell toxicity and depletion of CREB binding protein (CBP) or its recruitment into ...
Haibing Jiang +7 more
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Huntington disease: Advances in the understanding of its mechanisms
Clinical Parkinsonism & Related Disorders, 2020 Huntington disease (HD) is a devastating monogenic autosomal dominant disorder. HD is caused by a CAG expansion in exon 1 of the gene coding for huntingtin, placed in the short arm of chromosome 4.
Emilia M. Gatto +5 more
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Advanced Science, EarlyView.Herein we report a boron‐based pyrazole, (Borsantrazole ‐ a small molecule that selectively targets oxidative stress) that significantly increases survival, reduces weight loss, delays disease onset, and affects global protein changes in the SOD1‐G37R mouse model of ALS.
Nitesh Sanghai +9 more
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Neurobiology of Disease, 2002 Huntington's disease, with its dominant loss of striatal neurons, is triggered by an expanded glutamine tract in huntingtin. To investigate a proposed role for increased activation of the apoptotic cascade in mutant huntingtin's trigger mechanism, we ...
Shobu Namura +7 more
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Structural Polymorphism of polyG Inclusions Revealed by In Situ Cryo‐Electron Tomography
Advanced Science, EarlyView.Correlative cryo‐electron tomography in primary cortical neurons and NIID mouse brain tissue reveals that polyG inclusions are interconnected ribbon‐like assemblies rather than canonical amyloid fibrils. Multiple compartment‐specific ribbon states show distinct 26S proteasome accessibility, while cytoplasmic ribbons contact and deform ER‐like ...
Yunwen Qian +12 more
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Huntingtin interacts with a family of WW domain proteins [PDF]
Human Molecular Genetics, 1998 The hallmark neuropathology of Huntington's disease (HD) is due to elongation of a polyglutamine segment in huntingtin, a novel approximately 350 kDa protein of unknown function. We used a yeast two-hybrid interactor screen to identify proteins whose association with huntingtin might be altered in the pathogenic process.
P W, Faber +5 more
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