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. Author manuscript; available in PMC: 2011 Jan 1.

Published in final edited form as: Mol Genet Metab. 2010 Jan;99(1):62–71. doi: 10.1016/j.ymgme.2009.08.002

Table 1.

Lysosomal Enzyme Activity in Mouse Tissue Lysates.

		5 weeks		10 weeks		30 weeks
		WT (n=5)	W392X (n=5)	WT (n=6)	W392X (n=5)	WT (n=4)	W392X (n=4)
α-Idua	Brain	8.4±1.7	bls	9.6±1.3	bls	11.3±0.4	bls
α-Idua	Liver	4.6±1.8	bls	7.1±1.0	bls	5.6±0.9	bls
β-Gluc	Brain	17.0±2.8	^#55.7±9.1	8.8±1.5	^*13.4±3.0	8.4±0.5	^#18.7±2.3
β-Gluc	Liver	154.5±34.9	^#372.0±88.2	119.8±12.5	^#394.0±42.6	117.4±4.2	^#635.8±29.8
β-Hex	Brain	2303.5±284.3	^#4397.1±736.0	431.3±52.0	^#1486.8±231.4	413.2±36.9	^#2087.5±151.1
β-Hex	Liver	435.7±159.5	^#3071.0±718.7	160.6±31.1	^#1547.3±309.2	140.5±10.3	^#2417.5±226.5

Data expressed as the mean ± SD of the enzymatic specific activity (nanomoles FMU released per milligram total protein per hour);

n = number of mice; bls = below level of sensitivity for assay.

^#

p < 0.001,

^*

p < 0.02.