| First Author | Casal ML | Year | 1998 |
| Journal | Lab Invest | Volume | 78 |
| Issue | 12 | Pages | 1575-81 |
| PubMed ID | 9881957 | Mgi Jnum | J:51808 |
| Mgi Id | MGI:1326957 | Citation | Casal ML, et al. (1998) Variant clinical course of mucopolysaccharidosis type VII in two groups of mice carrying the same mutation. Lab Invest 78(12):1575-81 |
| abstractText | Mucopolysaccharidosis type VII (MPS VII) is a lysosomal storage disease caused by a deficiency in beta-glucuronidase. The disease has been well-characterized in B6.C-H-2bml/ByBir-gus(mps/mps) mice, which have proven to be an excellent model for the study of treatment modalities of storage diseases. However, because of the progressive nature of the disease, the mice deteriorate rapidly, have a greatly decreased life span, and are infertile. To increase fitness, B6.C-H-2 bml/ ByBir-gus(mps/+) were interbred with normal C3H mice. This study compares clinical findings, fertility, longevity, and gross and microscopic findings in the original strain with those in the F2 generation of the interbred strain. |
