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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

Fig. 2.

Fig. 2

A-SCS activity measurement by LC-MS/MS. (A) Time dependency was measured in control line and patient line at 37°C for 0, 3, 6, 9 and 12 min with 50 mM Tris buffer pH 8.0, 5 mM MgCl2, 10 mM D4-succinate, 1 mM ATP, 1 mM CoA, oligomycin 2 µg/ml and homogenate protein concentration 1mg/ml. Activity linearity is shown within 9min. (B) Protein dependency was measured in control line for 6 min at different protein concentrations, 0, 0.5, 0.75, 1 and 1.25 mg/ml. Other conditions were same as (A). Linear for protein concentrations <1.25 mg/ml. (C) and (D) Apparent Km and Vmax of succinate were measured from two control lines with different D4-succinate concentrations, 0.37, 1.11, 3.33, 10 and 30 mM, and 1mg/ml homogenate incubated at 37°C for 6min. Other conditions were same as (A).