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 diseases. However, because of the progressive nature of the disease, the mice deteriorate rapidly, have a greatly decreased...
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