Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation.

By S. Waelter, A. Boeddrich, R. Lurz, E. Scherzinger, G. Lueder, Hans Lehrach, Erich E. Wanker. Molecular Biology of the Cell, 2001

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Molecular mechanisms and potential therapeutical targets in Huntington's disease. (2010, background): “Huntingtin aggregates are labeled with antibodies to ubiquitin (48, 148, 619) and contain several proteasome subunits and chaperones (290, 494).”
Stress granule assembly is mediated by prion-like aggregation of TIA-1. (2004, background): “Indeed, TIA-1 has been reported to be a component of huntingtin inclusions (Waelter et al., 2001).”
Pathways of cellular proteostasis in aging and disease (2018, background): “, 2002), as exemplified by the fact that cellular aggregate deposits persist although they are typically associated with ubiquitin (Lowe et al., 1988; Bence et al., 2001; Waelter et al., 2001).”
Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue (2010, result): “The relationship between SGs and neurodegenerative diseases has been suggested by previous experiments demonstrating that PrP, SMN and huntingtin colocalize with SG proteins in inclusions [26,27,28].”
ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats. (2002, background): “Because dysfunctions of UPS and the ER appear to be involved in the pathogenesis of many neurodegenerative diseases, ASK1 may be a potential therapeutic target for various neurodegenerative disorders represented by polyQ diseases.”