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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
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Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
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Molecular Strategies to Target Protein Aggregation in Huntington's Disease.

Olga D Jarosińska1,2, Stefan G D Rüdiger1,2

  • 1Cellular Protein Chemistry, Bijvoet Centre for Biomolecular Research, Utrecht University, Utrecht, Netherlands.

Frontiers in Molecular Biosciences
|December 6, 2021
PubMed
Summary

Huntington's disease (HD) treatments are emerging. New strategies target mutant huntingtin (mHTT) protein levels and aggregates for degradation, offering a path to cure HD and other neurodegenerative diseases.

Keywords:
aggregationhuntingtin (HTT)huntington’s diseasemRNA degradationprotein degradationprotein fibrilsprotein quality controlproteostasis

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder characterized by mutant huntingtin (mHTT) protein aggregation in nerve cells.
  • mHTT aggregation disrupts cellular functions, leading to neuronal dysfunction and death.
  • Currently, no definitive treatments exist for HD.

Purpose of the Study:

  • To review current mRNA-targeting approaches for HD.
  • To explore novel methods for degrading mHTT and its aggregates.
  • To provide a knowledge-based strategy for targeting neurodegenerative diseases at their origin.

Main Methods:

  • Review of mRNA targeting strategies, including antisense oligonucleotides and RNAi systems.
  • Summary of novel degradation techniques targeting mHTT aggregates via proteasomal and lysosomal pathways.
  • Discussion of proteolysis-targeting chimeras, Trim-Away, and autophagy-targeting strategies.

Main Results:

  • mRNA targeting offers a systematic approach to reduce HTT levels.
  • Novel degradation methods show promise for clearing mHTT aggregates.
  • Various molecular strategies are being developed to target disease at the molecular level.

Conclusions:

  • Targeting HTT levels presents a promising therapeutic avenue for HD.
  • Degradation of mHTT and aggregates via proteasomal and lysosomal systems offers innovative treatment possibilities.
  • These molecular strategies provide a foundation for developing cures for HD and other neurodegenerative conditions.