Mutant Huntingtin Aggregates
Mutant Huntingtin Aggregates - Related Videos
04:56
Detection of Aggregation-Prone Behavior in Mutant P53 V157F Breast Cancer Cells Using Multipoint Thioflavin T Fluorescence
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2025
Hs578T breast cancer cells harboring the p53 V157F mutation exhibit significantly higher Thioflavin T fluorescence compared to MCF7 cells, indicating enhanced protein aggregation. Multipoint fluorescence measurements improve detection accuracy and reliability in identifying β-sheet-rich aggregates, underscoring the importance of aggregation-prone p53 mutations in cancer research and the development of therapeutic strategies.
04:12
Protein Aggregate Formation Assay: A Method to Detect and Quantify Protein Aggregation in Cultured Cells upon Induction by Proteasome Inhibitor
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2025
In this video, we demonstrate a cell-based protein aggregation assay using proteasome inhibitors, which block proteasome activity, preventing misfolded, mutant proteins, fused to a fluorescent label, from undergoing ubiquitin-dependent proteasomal degradation, leading to their accumulation within the cell cytoplasm. The protein aggregates are then visualized and quantified by fluorescence microscopy.
11:12
Assay Development for High Content Quantification of Sod1 Mutant Protein Aggregate Formation in Living Cells
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Cited by 2 •
2017
We describe a method to quantify the aggregation of misfolded proteins. Our protocol details lentiviral induced stable cell line generation, automated confocal imaging, and image analysis of protein aggregates. As an illustrative application, we studied the effect of small molecules in promoting SOD1 aggregation in a time- and dose-dependent manner.
05:47
Imaging and Quantification of Protein Aggregates in Genetically Modified Drosophila Brains
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2025
This video demonstrates imaging and quantification of protein aggregates in genetically modified Drosophila brains. Brains are fixed, washed, and treated with an antifading agent before mounting. Images are captured using a confocal microscope and quantified to observe the transmission of mutant protein aggregates.
10:10
Aggregates for Concrete and Asphaltic Mixes
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2023
Source: Roberto Leon, Department of Civil and Environmental Engineering, Virginia Tech, Blacksburg, VA Concrete and asphalt are by far the most common construction materials used today. Concrete is a composite material consisting of cement, water, air, coarse aggregate, and fine aggregates. Fine aggregates are typically sands and coarse aggregates are natural or crushed rocks. Chemical admixtures to modify certain specific properties are also commonly used (i.e., superplasticizers to make the...
FAQs
Expanded polyglutamine sequences promote abnormal folding of mutant huntingtin, creating protein conformations that can associate into oligomers, or small assemblies of misfolded protein. These oligomers may then accumulate into larger intracellular inclusions. Tracking this progression helps investigators connect an initial sequence alteration with changes in protein organization and the cellular abnormalities observed in Huntington’s disease.
Aggregate accumulation can interfere with protein quality control, the cellular processes that manage damaged or misfolded proteins, and with intracellular transport. These disruptions may reduce a neuron’s ability to maintain protein balance and move essential components. Examining both systems therefore provides a broader view of how aggregate-associated molecular abnormalities could contribute to impaired neuronal function.
Comparing aggregate formation with neuronal dysfunction can help explain why some neuronal populations are more vulnerable than others in Huntington’s disease. The relevant evidence includes aggregate burden, altered protein quality control, transport disruption, and changes in neuronal function. This comparison links molecular pathology to cell-type-specific outcomes without treating aggregate presence alone as a complete explanation of disease progression.
Researchers combine aggregate formation assays, imaging methods, and cellular or animal models. Assays can follow the formation of assemblies, while imaging examines their intracellular accumulation and distribution. Cellular models support controlled investigation of molecular effects, whereas animal models provide context for disease-related changes. Using these approaches together helps connect aggregate behavior with neuronal consequences.
These methods can indicate whether mutant huntingtin forms oligomers or larger inclusions, how much accumulation occurs, and where assemblies appear within cells or model systems. Such measurements help investigators compare disease-related conditions and evaluate whether an intervention changes aggregate formation or accumulation. Interpretation is strongest when structural observations are considered alongside effects on protein quality control and neuronal function.
Aggregate studies provide experimental readouts for investigating disease mechanisms, potential biomarkers, and therapeutic strategies. Researchers can assess approaches intended to reduce mutant huntingtin production, improve its clearance, or limit harmful interactions. Changes in aggregate formation or accumulation can then be compared with cellular or animal outcomes, helping determine whether a candidate intervention addresses a relevant disease-associated process.