Table 2.

Gb₃ clearance in organs of wild-type mice, noninjected α-gal A knockout mice, and α-gal A knockout mice injected with rAAV-CAG-hAGA

Weeks	Group of mice	Gb3 levels, nmol/mg protein
		Liver	Kidney	Heart	Spleen	Lung	SI
4	Wild type	0.04  ± 0.0	1.03  ± 0.5	0.13  ± 0.1	0.0  ± 0.0	0.68  ± 0.0	0.21  ± 0.1
	Noninjected Fabry	3.06  ± 0.7	8.41  ± 1.5	0.85  ± 0.1	3.85  ± 0.5	4.36  ± 0.9	8.91  ± 2.7
	Injected Fabry	1.95  ± 0.5*	4.92  ± 0.4*	0.72  ± 0.1*	2.96  ± 0.2*	4.2  ± 0.2*	9.29  ± 0.7*
8	Wild type	0.15  ± 0.1	0.78  ± 0.3	0.09  ± 0.0	0.45  ± 0.0	0.33  ± 0.1	0.22  ± 0.1
	Noninjected Fabry	4.18  ± 1.2	12.7  ± 5.8	1.65  ± 0.2	9.43  ± 1.3	1.75  ± 0.6	20.1  ± 7.1
	Injected Fabry	0.21  ± 0.1*	1.52  ± 0.6*	0.22  ± 0.0†	0.55  ± 0.2†	1.23  ± 0.3*	9.01  ± 0.9*
12	Wild type	0.04  ± 0.0	0.84  ± 0.6	0.0  ± 0.0	0.47  ± 0.1	0.0  ± 0.0	0.14  ± 0.0
	Noninjected Fabry	3.06  ± 0.1	6.52  ± 1.7	0.84  ± 0.3	15.9  ± 6.6	3.37  ± 0.8	14.9  ± 1.4
	Injected Fabry	0.04  ± 0.0†	1.31  ± 0.5†	0.43  ± 0.0*	1.08  ± 0.2*	1.75  ± 0.7	8.99  ± 2.9*
24	Wild type	0.08  ± 0.0	2.31  ± 1.1	0.02  ± 0.0	0.31  ± 0.0	0.18  ± 0.0	0.39  ± 0.1
	Noninjected Fabry	4.18  ± 1.2	12.4  ± 1.2	2.24  ± 0.4	49.7  ± 10	4.85  ± 1.8	23.8  ± 4.9
	Injected Fabry	0.34  ± 0.1*	5.02  ± 2.5†	0.0  ± 0.0†	0.72  ± 0.1†	0.6  ± 0.2*	7.97  ± 2.6†

Weeks after injection of the vector are shown in the left column. Mean Gb₃ ± SEM is shown for four mice at each time point. SI, small intestine. 

^*P < 0.05 vs. noninjected Fabry mice. 

^† P < 0.01.