Table 1. IDUA activities, GAG concentrations, and hexosaminidase activities in tissues of NSGI and Het mice.

Iduronidase activity, (units/mg protein)
Tissue	NSGI, N = 10	Het, N = 12	P
Brain	0	1.75 ± 0.248	<0.001
Heart	0	4.84 ± 0.381	<0.001
Kidney	0	2.17 ± 0.481	<0.001
Liver	0	4.89 ± 2.51	<0.001
Lung	0	3.80 ± 0.480	<0.001
Muscle	0	1.47 ± 0.792	<0.001
Spleen	0	18.5 ± 3.88	<0.001
µg GAG/mg dry tissuea
Tissue	NSGI, N = 3	Het, N = 4	P
Brain	4.02 ± 0.178	3.53 ± 0.154	0.011
Heart	19.4 ± 2.76	0	<0.001
Kidney	15.7 ± 5.94	0.323 ± 0.645	0.003
Liver	26.6 ± 0.586	0	<0.001
Lung	19.9 ± 2.10	0	<0.001
Muscle	5.98 ± 2.91	0	0.008
Spleen	26.1 ± 6.68	0.805 ± 0.537	<0.001
Urine	1,910 ± 1,660	131 ± 171	0.138
nmol GAG/mg proteinb
Tissue	NSGI, N = 7	Het, N = 8	P
Heart	25.7 ± 12.8	8.63 ± 2.20	0.002
Kidney	19.6 ± 6.31	10.2 ± 4.01	0.004
Liver	28.9 ± 9.99	15.2 ± 8.40	0.013
Muscle	17.1 ± 5.27	20.1 ± 7.47	0.401
Hexosaminidase activity, (units/mg protein)
Tissue	NSGI, N = 7	Het, N = 8	P
Brain	3,182 ± 258	1,764 ± 446	<0.001

Ns are as listed with the exception of lung and spleen where the Ns are 3 for the NSGI and 4 for the Hets.

^aUrinary GAG is expressed in µg/mg creatinine.

^bnmol GAG are approximate and are based on the glucuronic acid standard.