The TRIM37 variants in Mulibrey nanism patients paralyze follicular helper T cell differentiation.

ABSTRACT: The Mulibrey (Muscle-liver-brain-eye) nanism caused by loss-of-function variants in TRIM37 gene is an autosomal recessive disorder characterized by severe growth failure and constrictive pericarditis. These patients also suffer from severe respiratory infections, co-incident with an increased mortality rate. Here, we revealed that TRIM37 variants were associated with recurrent infection. Trim37 FIN_major (a representative variant of Mulibrey nanism patients) and Trim37 knockout mice were susceptible to influenza virus infection. These mice showed defects in follicular helper T (T_FH) cell development and antibody production. The effects of Trim37 on T_FH cell differentiation relied on its E3 ligase activity catalyzing the K27/29-linked polyubiquitination of Bc

SUBMITTER: Gu W 

PROVIDER: S-EPMC10394018 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature