Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation.

@article{Birkenmeier1991IncreasedLS,
  title={Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation.},
  author={Edward H. Birkenmeier and J. Ellis Barker and Carole A. Vogler and John W. Kyle and William S. Sly and Babette Gwynn and Beth Levy and C Pegors},
  journal={Blood},
  year={1991},
  volume={78 11},
  pages={3081-92}
}
The gusmps/gusmps mouse has no beta-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic abnormalities are similar to those found in humans with severe MPS VII. Mutant mice are dysmorphic, dwarfed, and have a shortened life span. Pathologic findings include widespread lysosomal storage. To determine whether bone marrow transplantation (BMT) corrects these abnormalities, genetically identical mutant animals were given syngeneic bone… CONTINUE READING

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