Evidence map›Paper›PMID 41853947›Full record

ArticleMovement disorders : official journal of the Movement Disorder Society2026

Modulation of the Stress Granule Component Carhsp1 Mitigates Disease-Associated Deficits in Spinocerebellar Ataxia Type 3 Mouse Models.

Tiago Moreira-Gomes, Adriana Marcelo, Ana Teresa Rajado, Rafael G Costa, Ricardo Afonso-Reis, Cristiana R Madeira, Inês T Afonso, David V C Brito, Adriana A Vaz, Clévio Nóbrega

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Article in Movement disorders : official journal of the Movement Disorder Society, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

10 authors.

Tiago Moreira-Gomes *ABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Adriana Marcelo *ABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Ana Teresa RajadoABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Rafael G CostaABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Ricardo Afonso-ReisABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Cristiana R MadeiraABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Inês T AfonsoABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
David V C BritoABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Adriana A VazABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.
Clévio NóbregaABC-Ri, Algarve Biomedical Center Research Institute, Faro, Portugal.ORCID https://orcid.org/0000-0002-8312-5292

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundSpinocerebellar ataxia type 3 (SCA3) is a polyglutamine (polyQ) neurogenerative disorder that results from CAG trinucleotide repeat expansions in the ATXN3 gene, leading to toxic protein aggregate formation and cellular pathway dysfunction. The dysfunction of cellular pathways also correlates with stress responses, such as the formation of stress granules (SG). Recent research suggests that SG and their components contribute to polyglutamine disease pathogenesis.

methodsWe combined the analysis of two mouse models for SCA3, data from RNA sequencing, and postmortem brain tissue samples.

resultsThis study intended to clarify the role of the SG component, calcium-regulated heat-stable protein 1 (CARHSP1), in SCA3. Among several SG components, CARHSP1 was found to be upregulated genetically in SCA3. The downregulation of Carhsp1 reduced the density of mutant protein aggregates, while enhancing motor function and alleviating neuropathological defects in SCA3 mouse models.

conclusionsThe findings indicate that modulating CARHSP1 and possible other SG components may provide a molecular target for therapeutics in SCA3, emphasizing the role of SG in polyQ disorders. © 2026 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Indexed as

Heat-Shock ProteinsMachado-Joseph DiseaseMolecular ChaperonesStress GranulesAnimalsAtaxin-3BrainDisease Models, AnimalHumansMiceMice, TransgenicTrinucleotide Repeat ExpansionAtaxin-3Atxn3 protein, mouseHeat-Shock ProteinsHsbp1 protein, mouseMolecular ChaperonesCarhsp1CRISPR‐Cas9polyglutamine diseasesspinocerebellar ataxia type 3stress granules

Identifiers

PMID41853947
PMCPMC13307246

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.