Deoxyribonuclease 1 (Dnase1)-de”cient mice develop symptoms of Systemic lupus erythematosus (SLE). Here we analysed the renal histopathology of these animals in comparison to F1 hybrids of New Zealand black and white mice (NZB/W F1), an established model of SLE. Animals were divided into three groups according to the presence of anti-nuclear antibodies (ANA) and renal lesions. Groups 1a–1c were healthy, whereas group 2 and 3 were classified as lupus-prone and affected. Subendothelial and/or mesangial immune complex deposits, mesangial and endocapillary proliferation,haematoxylin bodies and platelet aggregation were detected in both mouse strains but were more severe in the NZB/W F1 mice. The lupus nephritis was classi”ed as a proliferating (WHO type III or IV), which appeared to be preceded by a mesangial form (WHO type II). Subclassification of the ANA revealed a high prevalence of anti-nucleosome antibodies in Dnase1-deficient mice, whereas NZB/W F1 mice developed autoantibodies against a broad range of chromatin constituents. Mapping of the murine Dnase1 gene locus to chromosome 16A1-3 did not coincide with one of the reported susceptibility loci in the NZB/W F1 model, although a reduced Dnase1 serum and urine activity has been described previously in these mice.