Table 3.

GALT enzymatic activity in fibroblasts exposed to arginine

GALT activity (μmol/h/g protein)
Cell line	No arginine	0.1 mM arginine	1 mM arginine
Control 1 (n = 3)	31.6 ± 12.9	45.6 ± 25.1	35.3 ± 21.3
Control 2 (n = 2)	52.9	46.1	42.9
CG 1 (n = 2)	n.d.	n.d.	n.d.
CG 2 (n = 2)	n.d.	n.d.	n.d.
CG 3 (n = 2)	n.d.	n.d.	n.d.

Two wildtype (controls 1 and 2) and three classic galactosemic fibroblasts derived from two p.Q188R/p.Q188R patients (cell lines CG1 and CG2) and a p.Q188R/p.Q188P patient (cell line CG3) were cultured in the absence and in the presence of supplemental arginine (0.1 mM and 1 mM arginine). Results are expressed as μmol per hour per gram protein and presented as mean ± SD or average for n = 2. Number of replicates is presented in brackets

n.d.: non-detectable