Table. HBG activity in organs after treatment and effect of treatment on GAG levels in liver and spleen

GenotypeTransplanted macrophagesDays after infusionHBG activity in organsGAG level in liverGAG level in spleen
LiverSpleenCSDSHACSDSHA
+/−(n = 5) 
 
— 1030 ± 73 1369 ± 459 0.05 ± 0.01 0.04 ± 0.01 0.01 ± 0.00 0.68 ± 0.19 0.14 ± 0.06 0.06 ± 0.02 
−/−(n = 5) 
 
— 0.84 ± 0.75 0.44 ± 0.39 1.92 ± 0.78 0.39 ± 0.07 2.00 ± 0.29 5.78 ± 2.70 1.36 ± 0.53 2.21 ± 1.00 
−/−(n = 4) Normal  7 32.9 ± 8.6 35.0 ± 22.3 nd nd nd nd nd nd 
−/−(n = 3) Normal 38 3.6 ± 1.5 2.3 ± 0.6 0.37 ± 0.09* 0.07 ± 0.01* 0.14 ± 0.06* 3.64 ± 1.04 0.54 ± 0.13* 0.28 ± 0.13 
−/−(n = 3) Transduced  7 28.5 ± 4.3 32.4 ± 7.3 nd nd nd nd nd nd 
−/−(n = 3) Transduced 38 3.9 ± 0.8 2.3 ± 0.8 0.41 ± 0.10 0.14 ± 0.03* 0.20 ± 0.07* 2.80 ± 0.50 0.53 ± 0.12* 0.24 ± 0.09* 
GenotypeTransplanted macrophagesDays after infusionHBG activity in organsGAG level in liverGAG level in spleen
LiverSpleenCSDSHACSDSHA
+/−(n = 5) 
 
— 1030 ± 73 1369 ± 459 0.05 ± 0.01 0.04 ± 0.01 0.01 ± 0.00 0.68 ± 0.19 0.14 ± 0.06 0.06 ± 0.02 
−/−(n = 5) 
 
— 0.84 ± 0.75 0.44 ± 0.39 1.92 ± 0.78 0.39 ± 0.07 2.00 ± 0.29 5.78 ± 2.70 1.36 ± 0.53 2.21 ± 1.00 
−/−(n = 4) Normal  7 32.9 ± 8.6 35.0 ± 22.3 nd nd nd nd nd nd 
−/−(n = 3) Normal 38 3.6 ± 1.5 2.3 ± 0.6 0.37 ± 0.09* 0.07 ± 0.01* 0.14 ± 0.06* 3.64 ± 1.04 0.54 ± 0.13* 0.28 ± 0.13 
−/−(n = 3) Transduced  7 28.5 ± 4.3 32.4 ± 7.3 nd nd nd nd nd nd 
−/−(n = 3) Transduced 38 3.9 ± 0.8 2.3 ± 0.8 0.41 ± 0.10 0.14 ± 0.03* 0.20 ± 0.07* 2.80 ± 0.50 0.53 ± 0.12* 0.24 ± 0.09* 

HBG activity is expressed as nmol/h/mg. GAGs levels in liver and spleen are expressed in μg/mg of wet tissue. The data presented are mean ± SD.

CS, chondroitin sulfate; DS, dermatan sulfate; HA, hyaluronic acid; nd, not determined.

*

Indicates significant reduction (P < .05, 2-samplet test) of GAGs in tissues.

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