Lethal autoimmune myocarditis in interferon-gamma receptor-deficient mice: enhanced disease severity by impaired inducible nitric oxide synthase induction

Circulation. 2001 Jan 2;103(1):18-21. doi: 10.1161/01.cir.103.1.18.

Abstract

Background: Interferon-gamma (IFN-gamma) is an essential cytokine in the regulation of inflammatory responses in autoimmune diseases. Little is known about its role in inflammatory heart disease.

Methods and results: We showed that IFN-gamma receptor-deficient mice (IFN-gammaR(-/-)) on a BALB/c background immunized with a peptide derived from cardiac alpha-myosin heavy chain develop severe myocarditis with high mortality. Although myocarditis subsided in wild-type mice after 3 weeks, IFN-gammaR(-/-) mice showed persistent disease. The persistent inflammation was accompanied by vigorous in vitro CD4 T-cell responses and impaired inducible nitric oxide synthase expression, together with evidence of impaired nitric oxide production in IFN-gammaR(-/-) hearts. Treatment of wild-type mice with the nitric oxide synthetase inhibitor N:-nitro-l-arginine-methyl-ester enhanced in vitro CD4 T-cell proliferation and prevented healing of myocarditis.

Conclusions: Our data provide evidence that IFN-gamma protects mice from lethal autoimmune myocarditis by inducing the expression of inducible nitric oxide synthase followed by the downregulation of T-cell responses.

MeSH terms

  • Adjuvants, Immunologic / biosynthesis
  • Animals
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / enzymology*
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / immunology
  • CD4-Positive T-Lymphocytes / cytology
  • CD4-Positive T-Lymphocytes / drug effects
  • CD4-Positive T-Lymphocytes / immunology
  • Cell Division / drug effects
  • Dose-Response Relationship, Drug
  • Enzyme Induction / genetics
  • Enzyme Inhibitors / pharmacology
  • Immunity, Cellular / drug effects
  • Immunity, Cellular / genetics
  • Immunity, Cellular / immunology
  • Immunohistochemistry
  • Inflammation / enzymology
  • Inflammation / immunology
  • Inflammation / pathology
  • Interferon gamma Receptor
  • Interferon-gamma / metabolism
  • Interferon-gamma / pharmacology
  • Male
  • Mice
  • Mice, Inbred BALB C
  • Mice, Mutant Strains
  • Myocarditis / complications
  • Myocarditis / enzymology*
  • Myocarditis / genetics
  • Myocarditis / immunology
  • Myocardium / immunology
  • Myocardium / pathology
  • Myosin Heavy Chains / immunology
  • NG-Nitroarginine Methyl Ester / pharmacology
  • Nitric Oxide / biosynthesis
  • Nitric Oxide Donors / pharmacology
  • Nitric Oxide Synthase / antagonists & inhibitors
  • Nitric Oxide Synthase / biosynthesis*
  • Nitric Oxide Synthase / genetics
  • Nitric Oxide Synthase Type II
  • Receptors, Interferon / deficiency*
  • Receptors, Interferon / genetics
  • Severity of Illness Index

Substances

  • Adjuvants, Immunologic
  • Enzyme Inhibitors
  • Nitric Oxide Donors
  • Receptors, Interferon
  • Nitric Oxide
  • Interferon-gamma
  • Nitric Oxide Synthase
  • Nitric Oxide Synthase Type II
  • Nos2 protein, mouse
  • Myosin Heavy Chains
  • NG-Nitroarginine Methyl Ester