The extrafollicular B cell response is a hallmark of childhood idiopathic nephrotic syndrome.

ABSTRACT: The efficacy of the B cell-targeting drug rituximab (RTX) in childhood idiopathic nephrotic syndrome (INS) suggests that B cells may be implicated in disease pathogenesis. However, B cell characterization in children with INS remains limited. Here, using single-cell RNA sequencing, we demonstrate that a B cell transcriptional program poised for effector functions represents the major immune perturbation in blood samples from children with active INS. This transcriptional profile was associated with an extrafollicular B cell response marked by the expansion of atypical B cells (atBCs), marginal zone-like B cells, and antibody-secreting cells (ASCs). Flow cytometry of blood from 13 children with active INS and 24 healthy donors confirmed the presence of an extrafollicular B cell response den

SUBMITTER: Al-Aubodah TA 

PROVIDER: S-EPMC10667257 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature