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Ids-ko mice of 9 (b) and 14 (c) months of age, derived from the first mucopolysaccharidosis (MPS) II murine model [19,218], compared to a wild-type mouse of 9 months of age (a). Ids-ko mice show the progressive worsening of coarse fur, as well as distorted facies and broadened snouts, as a result of sclerosis and enlargement of the bones of the skull [219].
