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Strain Detail

Strain information
Resource No. nbio016
Strain name BKO
Strain Nomenclature
Common name/Synonyms β-galactosidase knockout mice, GM1-gangliosidosis model mice
Strain types mutant
Background strain C57BL/6NCr Slc
Institution National Institutes of Biomedical Innovation, Health and Nutrition (NIBIOHN)(formerly, National Institute of Infectious Diseases)
Developer Junichiro Matsuda
Depositor Junichiro Matsuda
Conditions of distribution without conditions : contact us
Animal Health Report
Strain description Photo of BKO mice

GM1-gangliosidosis is one of the typical lysosomal storage diseases and neurological genetic diseases caused by lysosomal β-galactosidase (β-Gal) gene mutations. GM1-gangliosidosis is lethal and no effective therapy is available, so the disease is designated intractable diseases. We developed a β-galactosidase knockout  mouse (BKO), and demonstrated that the mouse is a useful GM1-gangliosidosis model by showing that the mouse dies by 10 months old with neurologic symptoms caused by GM1 accumulation in neurons (1, 2). We also developed mouse models for infantile and adult forms of GM1-gangliosidosis by genarating transgenic mice with human mutant β-Gal genes. These mouse models are different in age of onset and severity. Furthermore, we are studying therapeutic drugs based on a new mechanism, "chemical chaperones", for treating brains using mouse models (Tg/KO) for human juvenile forms of GM1-gangliosidosis (3).

References
  1. Matsuda J, Suzuki O, Oshima A, Ogura A, Noguchi Y, Yamamoto Y, Asano T, Takimoto K, Sukegawa K, Suzuki Y, Naiki M. (1997) β-Galactosidase-deficient mouse as an animal model for GM1-gangliosidosis. Glycoconj J 14:729-736.
  2. Itoh M, Matsuda J, Suzuki O, Ogura A, Oshima A, Tai T, Suzuki Y, Takashima S. (2001) Development of lysosomal storage in mice with targeted disruption of the β-galactosidase gene: a model of human GM1-gangliosidosis. Brain Dev 23:379-384.
  3. Matsuda J, Suzuki O, Oshima A, Yamamoto Y, Noguchi A, Takimoto K, Itoh M, Matsuzaki Y, Yasuda Y, Ogawa S, Sakata Y, Nanba E, Higaki K, Ogawa Y, Tominaga L, Ohno K, Iwasaki H, Watanabe H, Brady RO, Suzuki Y. (2003) Chemical chaperone therapy for brain pathology in GM1-gangliosidosis. Proc Natl Acad Sci U S A 100:15912-15917.
Resource information
Cryopreserved sperm In-house Genotype β-Gal (+/−)
Medium FERTIUP
From other institutions Genotype
Medium
Cryopreserved embryo In-house Mating System
Genotype β-Gal (+/−), β-Gal (−/−)
Medium EFS40
From other institutions Mating System
Genotype
Medium
Strain status / Availability Cryopreserved sperm Within 1 month
Cryopreserved embryo Within 1 month
Live mouse Approx. 2 months
Gene information
Mutation types knockout
Gene symbol Glb1
Gene name galactosidase, beta 1
Genotyping information Genotyping protocol for BKO mice (pdf).