Epitope-level profiling in children with mitochondrial disease reveals limitations in the antibacterial antibody repertoire.

ABSTRACT:

Introduction

Immunometabolic studies in mice have suggested the importance of oxidative phosphorylation (OXPHOS) in humoral immunity. However, there are important distinctions between murine and human immunity. Furthermore, translational studies on the role of OXPHOS in humoral immunity are nearly absent from the biomedical literature. Children with primary OXPHOS deficiency (i.e., mitochondrial disease, MtD), are an important patient population for demonstrating the functional effects of this bioenergetic defect on humoral immunity.

Methods

To define whether OXPHOS deficiency extended to human B cells, we performed extracellular flux analysis on lymphoblastoid B cell lines from children with MtD and controls (N = 4/group). To expand the immune phenotype of B cell OXPHOS def

SUBMITTER: Gordon-Lipkin EM 

PROVIDER: S-EPMC10330363 | biostudies-literature | 2023 Jun

REPOSITORIES: biostudies-literature