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Related Experiment Video

Updated: Feb 10, 2026

Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy

Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy

Published on: June 27, 2018

Forms and Phases in Huntingtin Protein Aggregation.

Michael Elbaum¹

¹Department of Chemical and Biological Physics, Weizmann Institute of Science, Rehovot, Israel.

|May 19, 2018

Summary

Huntingtin protein exon1 with polyQ extensions forms liquid-like condensates that nucleate amyloid filaments. This study visualizes the initial stages of this process, crucial for understanding Huntington's disease pathogenesis.

Area of Science:

Biochemistry
Cell Biology
Neuroscience

Background:

Huntington's disease is a neurodegenerative disorder linked to polyglutamine (polyQ) expansions in the huntingtin protein.
Amyloid-like filaments are a hallmark of protein aggregation in neurodegenerative diseases.
Liquid-like condensates are implicated in cellular organization and disease processes.

Purpose of the Study:

To investigate the nucleation process of amyloid-like filaments from huntingtin protein exon1 condensates.
To visualize the initial formation of these pathogenic protein aggregates.

Main Methods:

Utilized a combination of fluorescence microscopy and electron tomography.
Studied liquid-like condensates of huntingtin protein exon1 with polyQ expansions.

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Related Experiment Videos

Last Updated: Feb 10, 2026

Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy

Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy

Published on: June 27, 2018

Methods to Study Changes in Inherent Protein Aggregation with Age in Caenorhabditis elegans

Methods to Study Changes in Inherent Protein Aggregation with Age in Caenorhabditis elegans

Published on: November 26, 2017

Monitoring Cell-to-cell Transmission of Prion-like Protein Aggregates in Drosophila Melanogaster

Monitoring Cell-to-cell Transmission of Prion-like Protein Aggregates in Drosophila Melanogaster

Published on: March 12, 2018

Main Results:

Observed the nucleation of amyloid-like filaments directly from liquid-like condensates.
Provided high-resolution structural insights into the early stages of huntingtin aggregation.

Conclusions:

Liquid-like condensates of huntingtin protein exon1 serve as nucleation sites for amyloid filament formation.
Understanding this nucleation mechanism is vital for developing therapeutic strategies for Huntington's disease.