CORRELATION BETWEEN THE CONCENTRATION OF SERUM ANTI-TOPOISOMERASE-I AUTOANTIBODIES AND HISTOLOGICAL AND BIOCHEMICAL-ALTERATIONS IN THE SKINOF TIGHT-SKIN MICE
A. Hatakeyama et al., CORRELATION BETWEEN THE CONCENTRATION OF SERUM ANTI-TOPOISOMERASE-I AUTOANTIBODIES AND HISTOLOGICAL AND BIOCHEMICAL-ALTERATIONS IN THE SKINOF TIGHT-SKIN MICE, Cellular immunology, 167(1), 1996, pp. 135-140
Cutaneous hyperplasia observed in tight skin mice is due to a mutation
located on chromosome 2. While homozygous mice die in utero the heter
ozygotes survive. TSK syndrome is associated with the presence of auto
antibodies specific for scleroderma target autoantigens. The presence
of autoantibodies specific for topoisomerase I is characteristic of bo
th human and murine disease. We have generated two distinct genotypes
of mice, TSK/+ and +/+ with respect to the TSK trait by breeding TSK m
ice with immunodeficient mouse strains. Since the mutated gene of TSK
syndrome has not yet been cloned, only histological and biochemical cr
iteria were used for defining TSK genotype. In the F1 mice derived by
mating TSK/+ mice with RAG2-/-, J(H)-/-, Or C57BLvit/vit mice, we have
found a good correlation between the amount of serum anti-topoisomera
se I autoantibodies present and the histopathological and biochemical
alterations that are characteristic of TSK scleroderma-like syndrome.
(C) 1996 Academic Press, Inc.