DOCK11 is an X-linked guanine nucleotide-exchange factor (GEF) that activates CDC42, a small Rho GTPase central to actin cytoskeleton dynamics 1. Primary functions include facilitating filopodia formation 2, marginal zone B-cell development, and B-cell homeostasis. Loss-of-function DOCK11 mutations cause X-linked autoinflammatory disease with immune dysregulation. In patients with hemizygous variants, DOCK11 deficiency impairs CDC42 activation, resulting in abnormal T-cell and B-cell migration, aberrant actin polymerization, and defective Treg differentiation with reduced FOXP3 expression 1. These cellular abnormalities culminate in severe early-onset autoimmunity including cytopenia, systemic lupus erythematosus, and inflammatory bowel disease 3. Complete loss-of-function variants primarily associate with autoinflammation and recurrent infections, while missense variants predominantly manifest autoantibody-related autoimmune features 3. DOCK11 mutations also cause normocytic anemia with abnormal erythrocyte morphology through impaired CDC42-dependent hematopoiesis 2. Additionally, DOCK11 regulates hematopoietic stem cell function through CDC42 activation; excess DOCK11 accumulation causes cellular senescence and impaired self-renewal 4. Beyond immunity, DOCK11 facilitates hepatitis B virus persistence by enabling retrograde trafficking and cccDNA maintenance, making it a potential antiviral target 5.