Table II.

Six of seven the SD or β-mutant cell lines with their relative activities of Hex A and Hex S after treatment with PYR or NGT.

β-mutant	βC137Y/C137Y		βT150P/P417L		βG353R/IVS12-26G>A		βP417L/Δ16kb		βR505Q/IVS11+5G>A		βP504S Δ16kb
Isoforms	Hex A	Hex S	Hex A	Hex S	Hex A	Hex S	Hex A	Hex S	Hex A	Hex S	Hex A	Hex S
PYRA	4.7+0.1D	6.7±0.2D	2.7±0.9C	10.8±0.9D	1.5±0.1D	3.2±0.1D	1.6±0.1D	4.0±0.2D	3.7±0.5C	3.9±0.4D	1.9±0.1D	2.2±0.1C
NGTB	4.8±1.5C	4.7±0.1D	2.1±0.1D	10.3±1.9C	1.6±0.1D	1.0±0.1	1.5±0.1D	3.4±0.1D	4.6±0.1C	5.4±0.1D	4.7±0.1D	3.1±0.4D

^A3.0 μg/mL,

^B300 μg/mL,

^CP < 0.05.

^DP < 0.01.