Assessment of bone dysplasia by micro-CT and glycosaminoglycan levels in mouse models for mucopolysaccharidosis type I, IIIA, IVA, and VII

@article{Rowan2012AssessmentOB,
  title={Assessment of bone dysplasia by micro-CT and glycosaminoglycan levels in mouse models for mucopolysaccharidosis type I, IIIA, IVA, and VII},
  author={Daniel J. Rowan and Shunji Tomatsu and Jeffrey H. Grubb and Adriana Maria Monta{\~n}o and William S. Sly},
  journal={Journal of Inherited Metabolic Disease},
  year={2012},
  volume={36},
  pages={235-246}
}
Mucopolysaccharidoses (MPS) are a group of lysosomal storage diseases caused by mutations in lysosomal enzymes involved in degradation of glycosaminoglycans (GAGs). Patients with MPS grow poorly and become physically disabled due to systemic bone disease. While many of the major skeletal effects in mouse models for MPS have been described, no detailed analysis that compares GAGs levels and characteristics of bone by micro-CT has been done. The aims of this study were to assess severity of bone… CONTINUE READING

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