Beta3-integrin-deficient mice are a model for Glanzmann thrombasthenia showing placental defects and reduced survival

J Clin Invest. 1999 Jan;103(2):229-38. doi: 10.1172/JCI5487.


beta3 integrins have been implicated in a wide variety of functions, including platelet aggregation and thrombosis (alphaIIbbeta3) and implantation, placentation, angiogenesis, bone remodeling, and tumor progression (alphavbeta3). The human bleeding disorder Glanzmann thrombasthenia (GT) can result from defects in the genes for either the alphaIIb or the beta3 subunit. In order to develop a mouse model of this disease and to further studies of hemostasis, thrombosis, and other suggested roles of beta3 integrins, we have generated a strain of beta3-null mice. The mice are viable and fertile, and show all the cardinal features of GT (defects in platelet aggregation and clot retraction, prolonged bleeding times, and cutaneous and gastrointestinal bleeding). Implantation appears to be unaffected, but placental defects do occur and lead to fetal mortality. Postnatal hemorrhage leads to anemia and reduced survival. These mice will allow analyses of the other suggested functions of beta3 integrins and we report that postnatal neovascularization of the retina appears to be beta3-integrin-independent, contrary to expectations from inhibition experiments.

Publication types

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

MeSH terms

  • Animals
  • Antigens, CD / genetics*
  • Bleeding Time
  • Digestive System / pathology
  • Disease Models, Animal
  • Erythrocyte Count
  • Female
  • Fetus / abnormalities
  • Fetus / physiopathology
  • Fluorescent Antibody Technique
  • Gene Targeting / methods
  • Hemorrhage / pathology
  • Integrin beta3
  • Integrins / metabolism
  • Leukocyte Count
  • Mice
  • Mice, Knockout
  • Platelet Aggregation / genetics
  • Platelet Membrane Glycoproteins / genetics*
  • Thrombasthenia / genetics*
  • Uterus / pathology


  • Antigens, CD
  • Integrin beta3
  • Integrins
  • Platelet Membrane Glycoproteins