Induction of Skin Fibrosis in Mice Expressing a Mutated Fibrillin-1 Gene

Saito, Shinichiro; Nishimura, Hiroyuki; Phelps, Robert; Wolf, Imre; Suzuki, Mihsa; Honjo, Tasuku; Bona, Constantin A.

doi:10.1007/bf03401821

Cited by 33 publications

(23 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…B cell-deficient J H D Ϫ/Ϫ TSK/ϩ mice exhibited cutaneous hyperplasia that is indistinguishable from that of TSK/ϩ mice despite diminished anti-topoisomerase I Ab (11). However, other previous reports have shown an association between skin sclerosis and autoantibody production (17,40,50,53). Although reasons for these discrepancies are unclear, these findings suggest that the TSK phenotype is multifactorial rather than due to a single mutation in the fibrillin 1 gene.…”

Section: Discussionmentioning

confidence: 59%

Intercellular Adhesion Molecule-1 Deficiency Attenuates the Development of Skin Fibrosis in Tight-Skin Mice

Matsushita

Hasegawa

Matsushita

et al. 2007

The Journal of Immunology

View full text Add to dashboard Cite

The tight-skin (TSK/+) mouse, a genetic model for systemic sclerosis, develops cutaneous fibrosis. Although a fibrillin 1 gene mutation and immunological abnormalities have been demonstrated, the roles of adhesion molecules have not been investigated. To directly assess roles of adhesion molecules in skin fibrosis, TSK/+ mice lacking L-selectin and/or ICAM-1 were generated. The deficiency of ICAM-1, but not L-selectin, significantly suppressed (∼48%) the development of skin sclerosis in TSK/+ mice. Similarly, ICAM-1 antisense oligonucleotides inhibited skin fibrosis in TSK/+ mice. Although T cell infiltration was modest into the skin of TSK/+ mice, ICAM-1 deficiency down-regulated this migration, which is consistent with the established roles of endothelial ICAM-1 in leukocyte infiltration. In addition, altered phenotype or function of skin fibroblasts was remarkable and dependent on ICAM-1 expression in TSK/+ mice. ICAM-1 expression was augmented on TSK/+ dermal fibroblasts stimulated with IL-4. Although growth or collagen synthesis of TSK/+ fibroblasts cultured with IL-4 was up-regulated, it was suppressed by the loss or blocking of ICAM-1. Collagen expression was dependent on the strain of fibroblasts, but not on the strain of cocultured T cells. Thus, our findings indicate that ICAM-1 expression contributes to the development of skin fibrosis in TSK/+ mice, especially via ICAM-1 expressed on skin fibroblasts.

show abstract

Section: Discussionmentioning

confidence: 59%

Intercellular Adhesion Molecule-1 Deficiency Attenuates the Development of Skin Fibrosis in Tight-Skin Mice

Matsushita

Hasegawa

Matsushita

et al. 2007

The Journal of Immunology

View full text Add to dashboard Cite

show abstract

“…For example while the Tsk mouse results from a mutation of the fibrillin gene, the phenotype is known to be multifactorial (Saito et al, 2000). It includes collagen fibrils that are thicker (Sundberg, 1994), and more densely packed than controls (Menton and Hess, 1980), and that have altered macroscopic organization (Menton et al, 1978).…”

Section: Discussionmentioning

confidence: 99%

Viscoelastic properties of skin in Mov-13 and Tsk mice

Prete

Antoniucci

Hoffman

et al. 2004

Journal of Biomechanics

View full text Add to dashboard Cite

“…Lung abnormality in Tsk/Cmice is likely due to factors other than Th2 cytokines. In fact, Saito and others reported that lung emphysema may be controlled by a different gene that cosegregates with mutated Fbn-1 [31].…”

Section: Discussionmentioning

confidence: 95%

Therapeutic effect of CpG-enriched plasmid administration on the tight-skin mouse model of scleroderma

Shen

Ichino

Nakazawa

et al. 2005

Journal of Autoimmunity

View full text Add to dashboard Cite

Induction of Skin Fibrosis in Mice Expressing a Mutated Fibrillin-1 Gene

Abstract: The results suggest that, although cutaneous hyperplasia is due to mutated Fbn-1 gene, the TSK syndrome may be multifactorial.

Cited by 33 publications

References 34 publications

Intercellular Adhesion Molecule-1 Deficiency Attenuates the Development of Skin Fibrosis in Tight-Skin Mice

Intercellular Adhesion Molecule-1 Deficiency Attenuates the Development of Skin Fibrosis in Tight-Skin Mice

Viscoelastic properties of skin in Mov-13 and Tsk mice

Therapeutic effect of CpG-enriched plasmid administration on the tight-skin mouse model of scleroderma

Contact Info

Product

Resources

About