Systemic sclerosis & Raynaud's
Caspase 1 Enhances Transport and Golgi Organization Protein 1 Expression to Promote Procollagen Export From the Endoplasmic Reticulum in Systemic Sclerosis Contributing to Fibrosis
Authors' conclusion
TANGO1 is markedly increased in SSc fibroblasts and was found to be elevated in lung tissue in association with α-SMA-positive cells. TANGO1 expression is driven by inflammasome-dependent caspase 1 activation and is mediated by IL-1 and TGFβ downstream signaling.
Study context
- Population
- Primary lung fibroblast cell lines and lung biopsy samples from SSc patients and healthy controls
- Participants
- 15 vs 12
- Comparison
- SSc vs healthy lung fibroblasts and lung tissue
- SScn = 15
- Healthyn = 12reference
- Outcome
- Role of TANGO1 in SSc fibrosis
Main finding
As reported in the abstract
- SSc fibroblasts showed increased TANGO1 protein expression in culture
- TANGO1 colocalizes with α-SMA-positive cells in SSc lung tissue and is highly up-regulated in the neointima of SSc vessels
- TANGO1 expression depended on inflammasome activation of caspase 1
- TANGO1 expression also depended on IL-1 and TGFβ receptor signaling
- Decreased TANGO1 down-regulated export of larger cargos incl. collagen and laminin
- Reduced TANGO1 had no effect on smaller molecular weight cargoes, but elastin secretion was significantly reduced
Original publication
Caspase 1 Enhances Transport and Golgi Organization Protein 1 Expression to Promote Procollagen Export From the Endoplasmic Reticulum in Systemic Sclerosis Contributing to Fibrosis. Arthritis Rheumatol. Published 16 August 2023. doi:10.
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