Disrupting the IL-4 gene rescues mice homozygous for the tight-skin mutation from embryonic death and diminishes TGF-beta production by fibroblasts

Proc Natl Acad Sci U S A. 2002 Mar 19;99(6):3800-5. doi: 10.1073/pnas.052709999. Epub 2002 Mar 12.

Abstract

The TSK/TSK mutation is embryonic lethal; embryos have been reported to die at 7-8 days of gestational age. Crossing TSK/+, IL-4+/- mice revealed that disrupting one or both IL-4 alleles allowed survival of 29 and 47%, respectively, of TSK/TSK mice. These mice failed to develop cutaneous hyperplasia but did exhibit the emphysema that is found in TSK/+ mice. We showed that IL-4 stimulation of fibroblasts increased the level of transforming growth factor-beta (TGF-beta) mRNA and that lungs of TSK/+, IL-4-/- mice had substantially less TGF-beta mRNA than lungs of TSK/+, IL-4+/+ mice. Thus IL-4 seems to regulate the expression of TGF-beta in fibroblasts, providing an explanation for the absence of cutaneous hyperplasia in TSK/+, IL-4Ralpha-/- and TSK/+, TGF-beta+/- mice.

Publication types

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

MeSH terms

  • Animals
  • Collagen / biosynthesis
  • Crosses, Genetic
  • Embryo Loss / genetics*
  • Embryo Loss / metabolism
  • Female
  • Fibroblasts / metabolism*
  • Fibroblasts / pathology
  • Gene Deletion
  • Gene Expression Regulation
  • Half-Life
  • Homozygote*
  • Interleukin-4 / deficiency*
  • Interleukin-4 / genetics
  • Interleukin-4 / metabolism
  • Lung / metabolism
  • Lung / pathology
  • Male
  • Mice
  • Mice, Inbred Strains
  • Mutation / genetics*
  • Phenotype
  • Pulmonary Emphysema / genetics
  • Pulmonary Emphysema / metabolism
  • Pulmonary Emphysema / pathology
  • RNA Stability
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Skin / metabolism
  • Skin / pathology*
  • Suppression, Genetic / genetics
  • Survival Rate
  • Transforming Growth Factor beta / genetics
  • Transforming Growth Factor beta / metabolism*

Substances

  • RNA, Messenger
  • Transforming Growth Factor beta
  • Interleukin-4
  • Collagen