TABLE 1.

Residual ASL activities in 293T cells (co-)transfected with recombinant ASL-WT and transcript variants

Data were obtained under standard conditions (13.6 mm argininosuccinate) after at least three independent experiments. ASL activity in cells (co-)expressing ASL-WT or ASL-WT with EV is set to 100% in each transfection (7 μg of plasmid) or co-transfection (total 10.5 μg of plasmids, each 3.5 μg) under the same condition, respectively. NT, non-transfected cells; EV: empty vector.

293T whole cell extracts	NT	EV	ex2del	ex7del	WT	WT + EV	WT + EV + ex2del	WT + EV + ex7del	WT + ex2del + ex7del	ex2del + ex7del + EV
ASL activity in mIU/mg total proteina	1.2 ± 1.9	1.6 ± 2.2	3.2 ± 3.0	1.9 ± 1.4	830.2 ± 78.8	520.1 ± 297.5	307.5 ± 151.4	466.1 ± 232.7	305.3 ± 201.7	3.2 ± 1.5
ASL activity in % of ASL-WTa	0.4 ± 0.4	0.3 ± 0.2	0.5 ± 0.4	0.4 ± 0.2	100.0 ± 7.8	100.0 ± 2.6	62.5b ± 7.2	94.2 ± 9.4	55.5b ± 7.2	0.7 ± 0.3

^a Mean S.D. (in triplicate).

^b Significant difference compared with ASL-WT activity (p < 0.05).