(A) Mutations within all kindlin family members cause pathology in humans and mice. Patients shown here have Kindler syndrome mutations within Kindlin-1. Kindlin-1 is mutated in Kindler syndrome, a skin-blistering disease caused by impaired actin attachment in basal keratinocytes. Kindlin-1 is expressed in epithelial cells. Kindlin-2 is important in early embryogenesis and is expressed in mesenchymal cells. Kindlin-3 is mutated in leukocyte adhesion deficiency, type III, a disease associated with infections, poor wound healing, and cutaneous bleeding. Kindlin-3 is expressed in hematopoietic cells. (B) Kindlin and Talin bind to the cytoplasmic tail of integrin receptors (left) and induce a conformational change in this receptor that permits the binding to its ligand. Kindlin also regulates the recycling of CD39 and CD73 receptors (right), thereby affecting the expression of these receptors on the cell membrane. The images in panel A were taken from Kloeker et al3 (left) and Siegel et al4 (right).
