Table 2.

Enzyme activities of ID2S, GALN, and ARSB in disease-affected individuals.

Sample	ID	Enzyme activity			Relative enzyme activity
		ID2S	GALN	ARSB	ID2S/GALN	GALN/ARSB	ARSB/ID2S	ID2S/GALN	GALN/ARSB	ARSB/ID2S
		(μmol/h/L)				(Ratio)			(%QCaverage)
MPS II	1	0.7	1.8	8.7	0.40	0.21	12	14	1.2	6
	2	0.2	1.1	3.7	0.15	0.30	23	5	1.7	11
	3	0.4	2.0	6.7	0.20	0.30	17	7	1.8	8
	4	0.8	1.4	4.5	0.62	0.30	5	22	1.8	3
	5	0.4	1.8	7.1	0.23	0.26	17	8	1.5	8
	6	0.4	1.3	10.0	0.32	0.13	24	11	0.8	11
MPS IVA	1	37.5	0.4	4.5	89	0.09	0.12	3118	0.55	0.06
	2	17.3	0.2	7.6	79	0.03	0.44	2752	0.17	0.21
	3	17.6	0.2	5.5	80	0.04	0.31	2795	0.24	0.15
MPS VI	1	24.9	2.4	0.3	10	9	0.01	357	51	0.005
QC High		12.2	4.1	24.0	3.01	17	197	105	99	93
QC Middle		6.4	1.9	11.3	3.32	17	177	116	100	84
QC Low		0.9	0.4	2.2	2.26	17	260	79	100	123
QCaverage		NA	NA	NA	2.86	17	212	100	100	100

Each QC High, Middle, and Low DBS contains 100, 50, and 5% of leukocytes with the same hematocrit values to normal blood.

All disease-affected individuals received enzyme replacement therapy at the time of sample collection.