Table 2.

Hurler syndrome mouse model in the literature and the present study

Deleted locus	Properties	Reference
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