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. Author manuscript; available in PMC: 2018 Mar 1.
Published in final edited form as: Mol Genet Metab. 2016 Nov 12;120(3):213–222. doi: 10.1016/j.ymgme.2016.11.005

Table 2.

ATP-SCS and GTP-SCS activity in control and patient fibroblast cell lines

ATP-SCS
nmol/min/mg protein 		Control line 1 Control line 2 Control line 3 Patient S2 line 1 Patient S2 line 2 Patient S1
Mean ± SD 1.02±0.45 0.98±0.32 1.04±0.50 0.089±0.052 0.177±0.057 0.096±0.0013
Replicates 15 13 10 12 7 2
Mean ± SD of 3
control lines 		1.01±0.029
Percentage of
control lines 		8.78% 17.52% 9.49%
GTP-SCS
nmol/min/mg protein 		Control line 1 Control line 2 Control line 3 Patient S2 line 1 Patient S2 line 2 Patient S1
Mean ± SD 8.58±1.90 7.60±2.45 10.91±3.85 13.91±3.42 10.51±2.41 12.07±6.92
Replicates 13 9 7 9 9 3
Mean ± SD of 3
control lines 		9.03±1.70
Percentage of
control lines 		154.04% 116.34% 133.70%