Article
Variant clinical course of mucopolysaccharidosis type VII in two groups of mice carrying the same mutation.
Laboratory investigation; a journal of technical methods and pathology - 1 Dec 1998
Casal M L, Wolfe J H
Abstract excerpt
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 disease...
Topics
- Animals
- Bone and Bones
- Brain
- Crosses, Genetic
- Disease Progression
- Female
- Genetic Carrier Screening
- Genotype
- Glucuronidase
- Glycosaminoglycans
- Liver
- Male
- Mice
- Mice, Inbred C3H
- Mice, Mutant Strains
- Mucopolysaccharidosis VII
- Muscle, Skeletal
- Pedigree