The abnormal lpr double-negative T cell fails to proliferate in vivo

ES Sobel, VN Kakkanaiah, RG Rapoport… - Clinical immunology and …, 1995 - Elsevier
Mice homozygous for the autosomal recessive gene lpr develop marked lymphadenopathy
and a systemic autoimmune disease resembling human systemic lupus erythematosus. The
enlarged nodes are dominated by T cells with an unusual surface phenotype: dull Thy-1+,
dull CD3+, CD4-, CD8-, B220+ (double-negative T cells or DNTs). Despite their massive
accumulation in vivo, these cells fail to proliferate in response to conventional T-cell
mitogens in vitro. The identification of the lpr mutation as a defect in the Fas apoptosis …