Sjögren's disease & overlap diseases
Diminished SUV3 expression and its functional implications in the interferon (IFN)-enriched monocyte subset of childhood Sjögren’s disease
An erratum to this article was published (Rheumatology (Oxford), 4 Feb 2026 ↗)
Authors' conclusion
SUV3 is the key driver for defective innate immune functions through mt-dsRNA-mediated PKR activation, which enhances cellular stress, mitochondrial dysfunction and inflammatory signatures, uncovering a novel mechanism in cSjD pathogenesis.
Study context
- Population
- Monocytes (IFN-enriched CD14+ subset) from childhood Sjögren's disease patients; monocytic cells with SUV3 knockdown
- Comparison
- Childhood Sjögren's disease (cSjD) monocytes vs controls; SUV3 knockdown in monocytic cells
Methodological detailssingle-cell RNA sequencing, SUV3 knockdown in monocytic cells, formaldehyde crosslinking immunoprecipitation (fCLIP)-qPCR, transmission electron microscopy
- Single-cell RNA sequencing
- SUV3 knockdown in monocytic cells
- fCLIP-qPCR
- Transmission electron microscopy
Main finding
As reported in the abstract
- SUV3 knockdown led to accumulation of mitochondrial-dsRNAs (mt-dsRNAs) outside the mitochondria, interacting with protein kinase R (PKR)
- PKR activation triggered a type I IFN signature and upregulated proinflammatory cytokines linked to fatigue
- Transmission electron microscopy (TEM) revealed mt-dsRNAs in mitochondrial-derived vesicles and multi-vesicular bodies
- SUV3 depletion increased superoxide and ROS production and impaired ATP synthesis, migration and phagocytosis
- Defects partially or fully reversed by co-knockdown of PKR
Further findings
Frequency of dsRNA-positive monocytes
P < 0.002
Original publication
Diminished SUV3 expression and its functional implications in the IFN-enriched monocyte subset of childhood Sjögren’s disease. Rheumatology (Oxford). Published 23 April 2025. doi:10.
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