Neurological manifestations of knockout mice with beta-galactosidase deficiency.
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.
Matsuda
J
J
Department of Veterinary Science, National Institute of Health, Tokyo, Japan. jmatsuda@nih.go.jp
Suzuki
O
O
Oshima
A
A
Ogura
A
A
Naiki
M
M
Suzuki
Y
Y
D000818:Animals; D004195:Disease Models, Animal; D005260:Female; D016537:Gangliosidosis, GM1 / Q000235:genetics*; D005838:Genotype; D006720:Homozygote; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D008818:Mice, Neurologic Mutants; D016296:Mutagenesis / Q000502:physiology; D001616:beta-Galactosidase / Q000172:deficiency* / Q000235:genetics*
D001616:beta-Galactosidase