ABSTRACT
The scurfy mutation occurred spontaneously in the partially inbred MR stock at the Oak Ridge National Laboratory. Severely anemic animals may exhibit secondary cardiomegaly, pleural effusion, and mild icterus. The small thymus in scurfy mice retains a distinct cortex and medulla until the last stages of the disease when the cortex becomes depleted of lymphocytes. The scurfy mutation appears to cause a defect in thymic education of lymphocytes that results in autoimmune T-cell reactivity and disease. The cutaneous lesions in scurfy mice resemble those described in the early stages of progressive systemic sclerosis (scleroderma), and nude mice receiving scurfy thymic transplants often develop severe dermal sclerosis. Wiscott-Aldrich syndrome lymphocytes show defective expression of leukosialin, a cell-surface glycoprotein. Scurfy lesions are markedly similar to those described in experimental murine chronic graft-vs.-host disease. The scurfy mutation can be used to investigate thymic development, aberrant T-cell function, and immune-mediated skin disease.
