The characterization of a murine model of mucopolysaccharidosis II (Hunter syndrome)

Abstract

Mucopolysaccharidosis II (MPS II, Hunter syndrome in humans) is an X-linked inherited lysosomal storage disease caused by a deficiency in the lysosomal enzyme iduronate-2-sulfatase (I2S). I2S catalyses a step in the catabolism of glycosaminoglycans (GAGs) dermatan sulfate and heparan sulfate, and when it is deficient or absent GAGs accumulate in tissues and… (More)
DOI: 10.1007/s10545-007-0641-8

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@article{Garcia2007TheCO, title={The characterization of a murine model of mucopolysaccharidosis II (Hunter syndrome)}, author={A R Garcia and J. Pan and Justin C Lamsa and Joseph Muenzer}, journal={Journal of Inherited Metabolic Disease}, year={2007}, volume={30}, pages={924-934} }