A Spontaneous H2-Aa Point Mutation Impairs MHC II Synthesis and CD4⁺ T-Cell Development in Mice.

ABSTRACT: Major histocompatibility complex class II (MHC II) is an essential immune regulatory molecule that plays an important role in antigen presentation and T-cell development. Abnormal MHC II expression can lead to immunodeficiency, clinically termed as type II bare lymphocyte syndrome (BLS), which usually results from mutations in the MHC II transactivator (CIITA) and other coactivators. Here, we present a new paradigm for MHC II deficiency in mice that involves a spontaneous point mutation on H2-Aa. A significantly reduced population of CD4⁺ T cells was observed in mice obtained from the long-term homozygous breeding of autophagy-related gene microtubule-associated protein 1 light chain 3 β (Map1lc3b, Lc3b) knockout mice; this phenotype was not attributed to th

SUBMITTER: Zhao Y 

PROVIDER: S-EPMC8931304 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature