Latency-associated peptide prevents skin fibrosis in murine sclerodermatous graft-versus-host disease, a model for human scleroderma

J Invest Dermatol. 2003 Oct;121(4):713-9. doi: 10.1046/j.1523-1747.2003.12517.x.

Abstract

Murine sclerodermatous graft-versus-host disease (Scl GVHD), produced by transplanting B10.D2 bone marrow and spleen cells to lethally irradiated BALB/cJ mice, is a model for human scleroderma. Mice with Scl GVHD have skin thickening, lung fibrosis, cutaneous mononuclear cell infiltration, and upregulation of cutaneous transforming growth factor beta1 (TGF-beta1) and type I collagen mRNAs by day 21 after bone marrow transplantation. Elevated TGF-beta1 appears to be the critical cytokine driving fibrosis in Scl GVHD, which can be prevented with antibodies to TGF-beta administered early in disease. Here we demonstrate that we can also prevent skin thickening in mice with Scl GVHD with a naturally occurring antagonist to TGF-beta1, human latency-associated peptide (LAP). By quantitative real-time PCR analysis and immunostaining, LAP treatment also abrogates the upregulation of cutaneous TGF-beta1 and connective tissue growth factor mRNAs and type I collagen synthesis in Scl GVHD. In contrast to anti-TGF-beta antibodies, LAP at 4 ng total per mouse has no significant suppressive effect on cutaneous influx of T cells and monocytes or immune cell activation. LAP may be a potential new therapy in scleroderma and other TGF-beta-driven fibrosing disease that targets TGF-beta more specifically, without affecting systemic critical roles of TGF-beta on immune cell function.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Biomarkers
  • Collagen Type I / biosynthesis
  • Connective Tissue Growth Factor
  • Disease Models, Animal
  • Female
  • Fibrosis
  • Gene Expression / drug effects
  • Graft vs Host Disease / drug therapy*
  • Graft vs Host Disease / immunology
  • Graft vs Host Disease / pathology
  • Immediate-Early Proteins / genetics
  • Intercellular Signaling Peptides and Proteins / genetics
  • Mice
  • Mice, Inbred BALB C
  • Peptide Fragments / pharmacology*
  • Protein Precursors / pharmacology*
  • RNA, Messenger / metabolism
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / immunology
  • Scleroderma, Systemic / pathology
  • Skin / pathology*
  • Transforming Growth Factor beta / genetics
  • Transforming Growth Factor beta1
  • Up-Regulation / drug effects

Substances

  • Biomarkers
  • CCN2 protein, human
  • CCN2 protein, mouse
  • Collagen Type I
  • Immediate-Early Proteins
  • Intercellular Signaling Peptides and Proteins
  • Peptide Fragments
  • Protein Precursors
  • RNA, Messenger
  • TGFB1 protein, human
  • Tgfb1 protein, mouse
  • Transforming Growth Factor beta
  • Transforming Growth Factor beta1
  • Connective Tissue Growth Factor