CRB1-Associated Retinal Dystrophy Patients Have Expanded Lewis Glycoantigen-Positive T Cells

• DOI: 10.1167/iovs.64.13.6
• Publication Date: 2023-10-04T15:30:55Z
• Authors: Lude Moekotte; Jonas J. W. Kuiper; Sanne Hiddingh; Xuan-Thanh-An Nguyen; Camiel J. F. Boon; L. Ingeborgh van ...; Joke H. de Boer; Maria M. van Gend...
• Keywords: Dystrophy

Purpose: Eye inflammation may occur in patients with inherited retinal dystrophies (IRDs) and is seen frequently IRDs associated mutations the CRB1 gene. The purpose of this study was to determine types inflammatory cells involved IRDs, by deep profiling composition peripheral blood mononuclear a CRB1-associated IRD. Methods: This included 33 an IRD confirmed 32 healthy controls. A 43-parameter flow cytometry analysis performed on isolated from venous blood. FlowSOM manual Boolean combination gating were used identify quantify immune cell subsets. Results: Comparing controls revealed significant increase abundance circulating CD4+ T CD8+ that express sialyl Lewis X antigen. Furthermore, we detected decrease plasmacytoid dendritic IgA+CD24+CD38+ transitional B-cell subset Conclusions: Patients show marked changes leukocyte composition, affecting lymphocyte populations. These results implicate pathways disease manifestations IRDs.