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Strain Detail: BK mice

Strain information
Resource No. nbio018
Strain name BK
Strain Nomenclature
Common name/Synonyms
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

GM1-gangliosidosis is classified three forms: infantile, juvenile and adult, according to the age of onset or clinical manifestation. Each clinical form has a human β-Gal gene mutation. GalB mice express mutant human β-Gal R201C found in infantile form. BK mice were produced by introducing human β-Gal R201C gene into β-Gal KO (BKO) mice. This strain is useful for GM1-gangliosidosis juvenile form model and used for new therapy development by chemical chaperone.

Reference(s)
  1. 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, and Suzuki Y. (2003) Chemical chaperone therapy for brain pathology in GM1-gangliosidosis. Proc. Natl. Acad. Sci. U.S.A. 100(26):15912-7. [PMID:14676316]
Resource information
Cryopreserved sperm In-house Genotype Tg/+, β-Gal (+/-)
Medium FERTIUP
From other institutions Genotype
Medium
Cryopreserved embryo In-house Mating System
Genotype Tg/+, β-Gal (+/-), β-Gal (-/-)
Medium EFS40
From other institutions Mating System
Genotype
Method
Strain status / Availability Cryopreserved sperm Within 1 month
Cryopreserved embryo Within 1 month
Live animals Approx. 2 months
Gene information
Mutation types transgenic + knockout
Gene symbol Glb1
Gene name galactosidase, beta 1
Genotyping information