Characterization of intestinal immune responses in generalized human and murine lipodystrophy - PubMed
6 hours ago
- #Lipodystrophy
- #T cells
- #Intestinal inflammation
- Acquired generalized lipodystrophy (AGL) is a rare metabolic disorder often linked with autoimmunity.
- Study used mass cytometry and single-cell RNA-seq to analyze intestinal immune responses in AGL.
- A patient with AGL and Crohn's disease (AGLCD) showed oligoclonal expansion of T cells in the periphery and inflamed intestine.
- Lipodystrophic mice, used as a model for AGL, did not exhibit T cell expansion and were protected from colitis.
- Allogeneic fat transplantations reversed the defect in proinflammatory T cell development in mice, suggesting lipodystrophy alone does not cause T cell expansion.
- A T cell-intrinsic de novo NRAS mutation was identified, indicating somatic mosaicism may drive clonal T cell expansion and intestinal inflammation in AGLCD.