Activated P13Kδ disrupts germinal center GC T(fh)/GC B cell cross talk and B cell antibody coding  

作　　者：I.Maeve Rea 

机构地区：[1]School of Medicine,Dentistry and Biomedical Sciences,Queens University Belfast,97 Lisburn Road,Belfast BT71NN,UK

出　　处：《Cellular & Molecular Immunology》2019年第11期848-850,共3页中国免疫学杂志（英文版）

摘　　要：In a study by Priete and research groups from the USA and Italy,1 a mouse model with a mutation in the P13K gene that causes the activation of the P13K pathway was used to demonstrate the cellular and molecular mechanisms underlying the main biological and clinical features of the rare human immunodeficiency disease known as APDS or activated P13kδsyndrome.In humans,the overactivation of the P13Kδpathway causes recurrent respiratory infections with secondary bronchiectasis,lymphopenia,lymphoproliferation,and poor vaccination responses.2 The P13Kδmouse model has a gain-of-function(GOF)mutation in the P13Kδgene and shows lymphopenia,lymphoproliferation,and poor responses to vaccination,similar to the findings in human disease.These findings suggested that the P13Kδ-GOF mouse could be a useful tool to dissect the cellular and molecular mechanisms and pathways that contribute to the similar human disease.The authors then probed the immune response/s in the P13KδGOF mouse model using a variety of challenges and methods to identify where and how the P13Kδ-GOF-activated gene disturbs the cellular and molecular pathways of competent antibody formation and used comparisons with wild-type(WT)mice and normal human donors to help define the APDS phenotype and the P13Kδ-GOF mutant mouse model(MM).

关 键 词：P13K VACCINATION RESPIRATORY 

分 类 号：R73[医药卫生—肿瘤]