Table 2 Hurler syndrome mouse model in the literature and the present study

Exon 6

• Targeted disruption

[14], Clarke K

• No detectable IDUA enzyme activity

• Increased urinary GAGs

• Flattened facial profile

• Thickening of the digits

Exon 6

• Targeted disruption

[13], Ohmi K

• No phenotype data

Between exon 8 and 9

• Knock-in mouse that carries IDUA-W402X mutation

[15], Wang D

• MPS I-H

• No detectable IDUA enzyme activity

• Increased GAGs in the urine and tissues

From a part of exon 6 to a part of exon 9

• Targeted disruption

This study

• Increased urine GAGs excretion

• Increased BMD and BMC