Creatinine was quantified using commercially available standard and internal standard. Considering that creatinine is a molecule found in all urine samples (even in synthetic urine), the calibration curve needed to be prepared in water to avoid underestimating creatinine levels in samples. Moreover, to obtain the most accurate and precise quantitation possible, a 9-point calibration curve with concentrations ranging from 0 to 30 mM of creatinine was used. A quadratic curve with a weighing 1/x and the original value included were selected. Data processing was achieved with MassLynx/QuanLynx software version 4.2 SCN982 (Waters Corporation, Milford, MA, USA). Regarding lyso-Gb1 and analogs, including polycyclic analogs, quantitation was achieved using a 9-point calibration prepared in synthetic urine with a commercially available lyso-Gb1 standard. Considering that no standards or internal standards are available for lyso-Gb1 analogs, we performed a relative quantitation of these latter metabolites using the lyso-Gb1 calibration curve and internal standard. Similar to the creatinine, lyso-Gb1 quantitation was achieved using a quadratic calibration curve with a weighing 1/x. Data were also processed with the MassLynx/QuanLynx software version 4.2 SCN982 (Waters, Milford, MA, USA).
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