What does this research mean for the field?

The VCP R191Q variant is associated with a distinct pattern of astrocyte-predominant tau pathology, featuring both 3-repeat and 4-repeat tau, alongside typical TDP-43 pathology. Novelty: ClaimNovelty.NOVEL_FINDING. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

Investigate the neuropathological spectrum and tau aggregate characteristics in a patient harboring a heterozygous VCP R191Q variant.

August 28, 2026Neuropathology

Astrocyte‐Predominant Tau Pathology in a Patient With VCP R191Q Variant

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Investigate the neuropathological spectrum and tau aggregate characteristics in a patient harboring a heterozygous VCP R191Q variant.
Conducted a postmortem neuropathological examination of brain and spinal cord tissue from a heterozygous VCP R191Q carrier presenting with muscle weakness, frontotemporal dementia, and parkinsonism.
Characterized tau pathology using immunohistochemistry, confocal microscopy for 3-repeat and 4-repeat tau isoforms, and Western blot analysis.
Demonstrated coexisting FTLD-TDP type D pathology alongside marked astrocytic tau pathology extending from the lower brainstem throughout the spinal cord.
Identified colocalized 3-repeat (3R) and 4-repeat (4R) tau within astrocytes with 3R predominance, yielding an Alzheimer's disease-like Western blot profile distinct from other astrocytic tauopathies.

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Implication

Postmortem analysis demonstrates astrocyte-predominant tau pathology in a patient carrying the VCP R191Q variant, suggesting a broader neuropathological spectrum for VCP-related disease.

Cite This Study

Taniguchi et al. (2026) studied this question.

synapsesocial.com/papers/6a91466cd15324a1df3a9fd9 https://doi.org/10.1111/neup.70075

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Implication

Postmortem analysis demonstrates astrocyte-predominant tau pathology in a patient carrying the VCP R191Q variant, suggesting a broader neuropathological spectrum for VCP-related disease.

Astrocyte‐Predominant Tau Pathology in a Patient With VCP R191Q Variant

Key Points

Discussion

Implication

Cite This Study

Astrocyte‐Predominant Tau Pathology in a Patient With VCP R191Q Variant

Key Points

Discussion

Implication

Cite This Study

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