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Single-Cell RNA Sequencing Reveals Impaired CHIP-Mediated Heat Stress Response in SCA3 Pathogenesis.

Mi-Bo Tang^1,2,3, Shi-Feng Sheng⁴, Zheng-Wei Hu⁵

¹Department of Geriatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, 450000, Henan, China. tangmibohappy@126.com.

Molecular Neurobiology

|February 17, 2026

Summary

Spinocerebellar ataxia type 3 (SCA3) progression is driven by reduced Hsc70-interacting protein (CHIP) levels, which impair cellular stress response. Restoring CHIP function offers a potential therapeutic strategy for SCA3.

Keywords:

CHIP HSF1 Heat shock stress SCA3 Single-cell RNA sequencing

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Area of Science:

Neuroscience
Genetics
Molecular Biology

Background:

Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder caused by ATXN3 gene CAG repeat expansions.
Mutant Ataxin-3 aggregation leads to neuronal intranuclear inclusions and neurotoxicity, but underlying mechanisms and therapies remain elusive.
Cellular stress responses are implicated in SCA3 pathogenesis.

Purpose of the Study:

To elucidate the key pathophysiological cascades driving SCA3 progression.
To identify potential therapeutic targets by investigating cellular and molecular alterations in SCA3 models.

Main Methods:

Analysis of SCA3 cellular and transgenic mouse models.
Measurement of cell viability and thermolability.
Investigation of Hsc70-interacting protein (CHIP) localization and levels.
Single-Cell RNA Sequencing (ScRNA-seq) of cerebellar tissue.
Native gel electrophoresis to assess heat shock factor 1 (HSF1) trimerization.
Assessment of CHIP overexpression effects on SCA3 phenotypes.

Main Results:

SCA3 cells showed reduced viability and increased thermolability.
CHIP was sequestered in neuronal intranuclear inclusions in SCA3 mice, decreasing soluble CHIP levels.
ScRNA-seq revealed dysregulated cellular stress responses in SCA3 cerebellums.
Reduced trimerized HSF1 levels in SCA3 cells indicated impaired heat stress response.
CHIP overexpression ameliorated SCA3 phenotypes by restoring HSF1/DNAJB1 function and heat stress response.

Conclusions:

Depletion of soluble CHIP promotes SCA3 progression by disrupting the CHIP-HSF1/DNAJB1 axis.
Impaired cellular stress response due to reduced CHIP is a critical pathogenic mechanism in SCA3.
Modulating CHIP activity represents a promising therapeutic strategy for SCA3.