Neutrophils drive type I interferon production and autoantibodies in patients with Wiskott-Aldrich syndrome.

ABSTRACT:

Background

Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency caused by mutations in Wiskott-Aldrich syndrome protein (WASp), a key regulator of cytoskeletal dynamics in hematopoietic cells. A high proportion of patients experience autoimmunity caused by a breakdown in T- and B-cell tolerance. Moreover, excessive production of type I interferon (IFN-I) by plasmacytoid dendritic cells (pDCs) contributes to autoimmune signs; however, the factors that trigger excessive innate activation have not been defined.

Objective

Neutrophil extracellular traps (NETs) emerged as major initiating factors in patients with diseases such as systemic lupus erythematosus and rheumatoid arthritis. In this study we explored the possible involvement of aberrant neutrophil functions i

SUBMITTER: Cervantes-Luevano KE 

PROVIDER: S-EPMC6089666 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature