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C57BL/6.C-H-2[bm1]/ByBir-gus[mps]

eagle-i ID

http://eagle-i.itmat.upenn.edu/i/0000013d-cbdf-0553-2ed7-105480000000

Resource Type

  1. Mus musculus

Properties

  1. Resource Description
    MPS VII mice (C57BL/6.C-<i>H-2<sup>bml</sup></i>/ByBir-<i>gus<sup>mps/mps</sup></i>) are generated by inbreeding the carrier strain C57BL/6.C-<i>H-2<sup>bml</sup></i>/ByBir-<i>gus<sup>mps/+</sup></i>. This colony has been maintained since 1990 when carrier mice were purchased from The Jackson Laboratory.
  2. Related Disease
    Sly syndrome
  3. Related Disease
    lysosomal storage disease
  4. Related Publication or Documentation
    Selective neurodegeneration in murine mucopolysaccharidosis VII is progressive and reversible
  5. Related Publication or Documentation
    Variant clinical course of mucopolysaccharidosis type VII in two groups of mice carrying the same mutation
  6. Related Publication or Documentation
    Mucopolysaccharidosis type VII in the developing mouse fetus
  7. Related Publication or Documentation
    Sustained production of beta-glucuronidase from localized sites after AAV vector gene transfer results in widespread distribution of enzyme and reversal of lysosomal storage lesions in a large volume of brain in mucopolysaccharidosis VII mice
  8. Genetic Alteration(s)
    gus[mps]
  9. Phenotype Findings
    Premature death
  10. Phenotype Findings
    Neurodegeneration
  11. Phenotype Findings
    Storage lesions
  12. Phenotype Findings
    Facial dysmorphia
  13. Phenotype Findings
    Decreased body weight
  14. Phenotype Findings
    Decreased mobility
  15. Phenotype Findings
    β-glucuronidase enzymatic activity
  16. Phenotype Findings
    Vacuolation
  17. Location
    Wolfe Laboratory
 
RDFRDF
 
Provenance Metadata About This Resource Record
  1. workflow state
    Published
  2. contributor
    fcoldren
  3. created
    2013-04-02T13:50:33.063-04:00
  4. creator
    fcoldren
  5. modified
    2013-04-18T14:40:54.480-04:00

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