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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis.

GM1-gangliosidosis is a progressive neurological disease in humans caused by deficiency of lysosomal acid beta-galactosidase, which hydrolyses the terminal beta-galactosidic residue from ganglioside GM1 and other glycoconjugates. In this study, we generated a mouse model for GM1-gangliosidosis by gene targeting in embryonic stem cells. The mouse homozygous for the disrupted beta-galactosidase gene showed beta-galactosidase deficiency, presented with progressive spastic diplegia, and died of emaciation at 7-10 months of age. Pathologically, PAS-positive intracytoplasmic storage was observed in neuronal cells of various areas in the brain. Biochemical analysis revealed a marked accumulation of ganglioside GM1 and asialo GM1 in brain tissue. This animal model will be useful for pathogenetic analysis and therapeutic trial of human GM1-gangliosidosis.[1]


  1. Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis. Matsuda, J., Suzuki, O., Oshima, A., Ogura, A., Noguchi, Y., Yamamoto, Y., Asano, T., Takimoto, K., Sukegawa, K., Suzuki, Y., Naiki, M. Glycoconj. J. (1997) [Pubmed]
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