Muir-Torre-like syndrome in Fhit-deficient mice

Proc Natl Acad Sci U S A. 2000 Apr 25;97(9):4742-7. doi: 10.1073/pnas.080063497.

Abstract

To investigate the role of the Fhit gene in carcinogen induction of neoplasia, we have inactivated one Fhit allele in mouse embryonic stem cells and produced (129/SvJ x C57BL/6J) F(1) mice with a Fhit allele inactivated (+/-). Fhit +/+ and +/- mice were treated intragastrically with nitrosomethylbenzylamine and observed for 10 wk posttreatment. A total of 25% of the +/+ mice developed adenoma or papilloma of the forestomach, whereas 100% of the +/- mice developed multiple tumors that were a mixture of adenomas, squamous papillomas, invasive carcinomas of the forestomach, as well as tumors of sebaceous glands. The visceral and sebaceous tumors, which lacked Fhit protein, were similar to those characteristic of Muir-Torre familial cancer syndrome.

Publication types

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

MeSH terms

  • Acid Anhydride Hydrolases*
  • Adenoma / genetics
  • Animals
  • Carcinogens
  • Dimethylnitrosamine / analogs & derivatives
  • Female
  • Humans
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred Strains
  • Mice, Knockout
  • Neoplasm Proteins / deficiency
  • Neoplasm Proteins / genetics
  • Neoplasms, Multiple Primary / chemically induced
  • Neoplasms, Multiple Primary / genetics*
  • Neoplasms, Multiple Primary / pathology
  • Papilloma / genetics
  • Proteins / genetics*
  • Proteins / metabolism
  • Restriction Mapping
  • Sebaceous Gland Neoplasms / genetics
  • Stomach Neoplasms / chemically induced
  • Stomach Neoplasms / genetics
  • Stomach Neoplasms / pathology
  • Syndrome

Substances

  • Carcinogens
  • Neoplasm Proteins
  • Proteins
  • fragile histidine triad protein
  • nitrosobenzylmethylamine
  • Acid Anhydride Hydrolases
  • Dimethylnitrosamine