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2 protocols using 2h9 myristoylcarnitine c14

1

Comprehensive Lipid and Metabolite Analysis

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PC(12:0/13:0), PE(12:0/13:0), SM(d18:1/12:0) and Cer(d18:1/12:0) were purchased from Avanti Polar Lipids (Alabaster, AL, USA). Stable isotope labeled references were purchased from Cambridge Isotope Laboratories (Andover, MA, USA), including: Glycine (2-13C, 15N), L-Alanine (2,3,3,3-D4), L-Valine (D8), L-Leucine (5,5,5-D3), L-Methionine (methyl-D3), L-Phenylalanine (ring-13C6), L-Tyrosine (ring-13C6), L-Aspartic acid (2,3,3-D3), DL-Glutamic acid (2,4,4-D3), L-Ornithine: HCl (5,5-D2), L-Citrulline (5,5-D2) and L-Arginine (5-13C, 4,4,5,5-D4). Deuterium-labeled carnitine and acylcarnitines (NSK-B set) were purchased from Cambridge Isotope Laboratories (Tewksbury, MA, USA), including 2H3-Acetylcarnitine (C2), 2H3-Propionylcarnitine (C3), 2H3-Butyrylcarnitine (C4), 2H9-Isovalerylcarnitine (C5), 2H3-Octanoylcarnitine (C8), 2H9-Myristoylcarnitine (C14), and 2H3-Palmitoylcarnitine (C16). HPLC-grade ammonium acetate was purchased from Sigma-Aldrich (St. Louis, MO, USA), and HPLC-grade formic acid and acetonitrile were purchased from Thermo Fisher Scientific (Waltham, MA, USA). All solutions were prepared by LC-MS ultra-pure water.
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2

Acylcarnitine and Amino Acid Analysis

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Plasma acylcarnitines and amino acids were measured and analyzed by liquid chromatography and tandem mass spectrometry (LC-MS/MS) on an Agilent 6460 Triple Quadrupole Mass Spectrometer by the Mount Sinai Biochemical Genetic Testing Lab (now Sema4), as previously described (Le et al. 2014; Leandro et al. 2019; Ranea-Robles et al. 2020) . The L-carnitine internal standard ( 2 H 9 -carnitine) and the acylcarnitine internal standard mix containing 2 H 9 -carnitine, 2 H 3 -acetylcarnitine (C2), 2 H 3 -propionylcarnitine (C3), 2 H 3 -butyrylcarnitine (C4), 2 H 9 -isovalerylcarnitine (C5), 2 H 3 -octanoylcarnitine (C8), 2 H 9myristoylcarnitine (C14), and 2 H 3 -palmitoylcarnitine (C16), were from Cambridge Isotope Laboratories (Tewksbury, MA, USA).
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