Blood and urine samples were collected to measure the levels of 26 Acylcarnitines (ACs) and 76 urine organic acids (UOA). Whole blood and serum were collected in serum tubes and stored between 2 and 8 °C. Fresh urine samples were collected in a test tube and stored frozen. All reagents and internal standards used for LC–MS/MS and for GC–MS were as previously described [15 (link)].
The ACs, extracted and derivatized to butyl esters as previously described [15 (link), 16 (link)], were analyzed on an API 4000 triple quadrupole mass spectrometer (Applied Biosystems-Sciex, Toronto, Canada) coupled with the high performance liquid chromatograph Agilent 1100 series (Agilent Technologies, Waldbronn, Germany).
Creatinine concentration of urine samples was determined by using automatic analysis system BM/Hitachi 904. Organic acids were extracted from urine and analysed on a GC–MS system including an Agilent 7890A (Agilent Technologies, Santa Clara, CA, USA) gas chromatograph and an Agilent 5975C mass spectrometer. Extraction and analysis were performed as previously described [17 ]; the concentration of organic acids was normalized to the creatinine concentration of the urine sample and expressed as mmol organic acid/mol creatinine.
The ACs, extracted and derivatized to butyl esters as previously described [15 (link), 16 (link)], were analyzed on an API 4000 triple quadrupole mass spectrometer (Applied Biosystems-Sciex, Toronto, Canada) coupled with the high performance liquid chromatograph Agilent 1100 series (Agilent Technologies, Waldbronn, Germany).
Creatinine concentration of urine samples was determined by using automatic analysis system BM/Hitachi 904. Organic acids were extracted from urine and analysed on a GC–MS system including an Agilent 7890A (Agilent Technologies, Santa Clara, CA, USA) gas chromatograph and an Agilent 5975C mass spectrometer. Extraction and analysis were performed as previously described [17 ]; the concentration of organic acids was normalized to the creatinine concentration of the urine sample and expressed as mmol organic acid/mol creatinine.
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