Mechanism of cytoskeleton-related regulation on the primary immunodeficiency diseases

Abstract:

Primary immunodeficiency disease (PID) is a group of immune disorder caused by gene mutation. It is clinically manifested as recurrent and severe infections especially by specific pathogenic microorganism and often accompanied by auto-immune and high susceptibility to tumors. Cytoskeleton is a system of filaments or fibers that maintains cell shape, support cell’s movement and regulates multiple cellular functions, mainly composed of microtubules, microfilaments and intermediate fibers. On the background of PID and taken Wiskott-Aldrich syndrome (WAS) and DOCK8 deficiency disease as examples, we reviewed on the regulation of WAS protein, DOCK8 as cytoskeleton proteins in cellular immunity, humoral immunity, immune metabolism and their role in nuclear regulation. In addition, we also addressed currently unsolved scientific questions.