Neurological manifestations of knockout mice with beta-galactosidase deficiency

Brain Dev. 1997 Jan;19(1):19-20. doi: 10.1016/s0387-7604(96)00077-0.

Abstract

We succeeded in producing the beta-galactosidase-deficient knockout mouse by gene targeting in embryonic stem cells. The mutant mice developed progressive spastic diplegia within a few months after birth, and died of emaciation at 7-10 months of age. This is an authentic murine model of human GMI-gangliosidosis, and is useful for studies of its pathogenesis and treatment.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Disease Models, Animal*
  • Female
  • Gangliosidosis, GM1 / genetics*
  • Genotype
  • Homozygote
  • Male
  • Mice
  • Mice, Knockout*
  • Mice, Neurologic Mutants
  • Mutagenesis / physiology
  • beta-Galactosidase / deficiency*
  • beta-Galactosidase / genetics*

Substances

  • beta-Galactosidase