TABLE 3.

Residual ASL activities in 293T cells co-transfected with recombinant ASL-WT, mutant p.E189G, and transcript variants

Data were obtained under standard conditions (13.6 mm argininosuccinate) after three independent experiments. ASL activity in cells co-expressing ASL-WT with EV is set to 100% in each co-transfection (total 7 μg of plasmids, each 3.5 μg) under the same conditions. All measurements were in triplicate.

293T whole cell extracts	WT + EV	WT + ex2del	WT + ex7del	E189G + EV	E189G + ex2del	E189G + ex7del	WT + E189G
Mean ASL activity in mIU/mg total protein (mean ± S.D.)	450.2 ± 38.3	286.1 ± 5.5	363.5 ± 15.0	389.1 ± 30.8	78.4 ± 26.4	257.2 ± 10.5	883.8 ± 80.0
Mean ASL activity in % of ASL-WT + EV (mean ± S.D.)	100.0 ± 7.1	61.9a ± 5.8	88.5 ± 11.5	88.4 ± 11.9	16.6a ± 5.3	57.5a ± 3.2	197.6 ± 8.9

^a Significant differences compared with ASL-WT activity (p < 0.05).