Table 1.

Sulphamidase uptake in MPS-IIIA MEFs

Transfection In Hepa cells	Empty	SGSH		IDSspSGSH		IDSspSGSH-ApoB
M6P in conditioned medium	−	−	+	−	+	−	+
Activity in recipient MPS-IIIA MEFs (nmol/17 h/mg)	1.39 (±0.14)	18.05 (±0.18)	5.59 (±0.50)	16.85 (±0.55)	5.40 (±0.63)	16.07 (±0.44)	11.15 (±0.65)

Sulphamidase activities (expressed as nmol/17 h/mg) were measured in MPS-IIIA MEFs cells grown for 24 h with conditioned media (containing 20 nmol/17 h/ml of sulphamidase) derived from Hepa transfected with wild-type, partially modified or fully modified sulphamidase encoding plasmids. As control MPS-IIIA MEFs were also incubated with conditioned medium derived from Hepa cells transfected with an empty vector. Conditioned media were incubated with MEFs in absence or in presence of M6P (5 mM). Values are means ± SEM of triplicate experiments.