Table 1

Blood spot acylcarnitine analysis by the butanol-HC derivatized method using LC-MS/MS.

Acylcarnitine SpeciesConcentration (µmol/L)Reference Interval (µmol/L)
Free carnitine (C0)7.08 (−)8.8–55
Acetylcarnitine (C2)8.457.5–59
Propionylcarnitine (C3)1.070–6.75
Malonylcarnitine (C3-DC)0.120–0.32
Iso-/Butyrylcarnitine (C4)0.250–0.55
Methylmalonyl-/succinylcarnitine (C4-DC)0.650–0.85
Isovaleryl-/2-methylbutyrylcarnitine (C5)0.200–0.65
Tiglylcarnitine (C5:1)0.050–0.22
Glutarylcarnitine (C5-DC)0.410–0.65
3-OH-isovalerylcarnitine (C5-OH)0.180–0.44
Hexanoylcarnitine (C6)0.080–0.26
Octanoylcarnitine (C8)0.100–0.27
Decanoylcarnitine (C10)0.100–0.21
Decenoylcarnitine (C10:1)0.080–0.18
Dodecanoylcarnitine (C12)0.660–1.01
Tetradecanoylcarnitine (C14)0.490–1.01
Tetradecenoylcarnitine (C14:1)0.240–0.65
Hexadecanoylcarnitine (C16)15.97 (+)0–7.19
3-OH-hexadecanoylcarnitine (C16-OH)0.120–0.33
Octadecanoylcarnitine (C18)3.53 (+)0–2.52
Octadecenoylcarnitine (C18:1)4.84 (+)0–3.16
3-OH-octadecanoylcarnitine (C18-OH)0.050–0.42
Acylcarnitine SpeciesConcentration (µmol/L)Reference Interval (µmol/L)
Free carnitine (C0)7.08 (−)8.8–55
Acetylcarnitine (C2)8.457.5–59
Propionylcarnitine (C3)1.070–6.75
Malonylcarnitine (C3-DC)0.120–0.32
Iso-/Butyrylcarnitine (C4)0.250–0.55
Methylmalonyl-/succinylcarnitine (C4-DC)0.650–0.85
Isovaleryl-/2-methylbutyrylcarnitine (C5)0.200–0.65
Tiglylcarnitine (C5:1)0.050–0.22
Glutarylcarnitine (C5-DC)0.410–0.65
3-OH-isovalerylcarnitine (C5-OH)0.180–0.44
Hexanoylcarnitine (C6)0.080–0.26
Octanoylcarnitine (C8)0.100–0.27
Decanoylcarnitine (C10)0.100–0.21
Decenoylcarnitine (C10:1)0.080–0.18
Dodecanoylcarnitine (C12)0.660–1.01
Tetradecanoylcarnitine (C14)0.490–1.01
Tetradecenoylcarnitine (C14:1)0.240–0.65
Hexadecanoylcarnitine (C16)15.97 (+)0–7.19
3-OH-hexadecanoylcarnitine (C16-OH)0.120–0.33
Octadecanoylcarnitine (C18)3.53 (+)0–2.52
Octadecenoylcarnitine (C18:1)4.84 (+)0–3.16
3-OH-octadecanoylcarnitine (C18-OH)0.050–0.42

(−): below reference range; (+): above reference range.

Table 1

Blood spot acylcarnitine analysis by the butanol-HC derivatized method using LC-MS/MS.

Acylcarnitine SpeciesConcentration (µmol/L)Reference Interval (µmol/L)
Free carnitine (C0)7.08 (−)8.8–55
Acetylcarnitine (C2)8.457.5–59
Propionylcarnitine (C3)1.070–6.75
Malonylcarnitine (C3-DC)0.120–0.32
Iso-/Butyrylcarnitine (C4)0.250–0.55
Methylmalonyl-/succinylcarnitine (C4-DC)0.650–0.85
Isovaleryl-/2-methylbutyrylcarnitine (C5)0.200–0.65
Tiglylcarnitine (C5:1)0.050–0.22
Glutarylcarnitine (C5-DC)0.410–0.65
3-OH-isovalerylcarnitine (C5-OH)0.180–0.44
Hexanoylcarnitine (C6)0.080–0.26
Octanoylcarnitine (C8)0.100–0.27
Decanoylcarnitine (C10)0.100–0.21
Decenoylcarnitine (C10:1)0.080–0.18
Dodecanoylcarnitine (C12)0.660–1.01
Tetradecanoylcarnitine (C14)0.490–1.01
Tetradecenoylcarnitine (C14:1)0.240–0.65
Hexadecanoylcarnitine (C16)15.97 (+)0–7.19
3-OH-hexadecanoylcarnitine (C16-OH)0.120–0.33
Octadecanoylcarnitine (C18)3.53 (+)0–2.52
Octadecenoylcarnitine (C18:1)4.84 (+)0–3.16
3-OH-octadecanoylcarnitine (C18-OH)0.050–0.42
Acylcarnitine SpeciesConcentration (µmol/L)Reference Interval (µmol/L)
Free carnitine (C0)7.08 (−)8.8–55
Acetylcarnitine (C2)8.457.5–59
Propionylcarnitine (C3)1.070–6.75
Malonylcarnitine (C3-DC)0.120–0.32
Iso-/Butyrylcarnitine (C4)0.250–0.55
Methylmalonyl-/succinylcarnitine (C4-DC)0.650–0.85
Isovaleryl-/2-methylbutyrylcarnitine (C5)0.200–0.65
Tiglylcarnitine (C5:1)0.050–0.22
Glutarylcarnitine (C5-DC)0.410–0.65
3-OH-isovalerylcarnitine (C5-OH)0.180–0.44
Hexanoylcarnitine (C6)0.080–0.26
Octanoylcarnitine (C8)0.100–0.27
Decanoylcarnitine (C10)0.100–0.21
Decenoylcarnitine (C10:1)0.080–0.18
Dodecanoylcarnitine (C12)0.660–1.01
Tetradecanoylcarnitine (C14)0.490–1.01
Tetradecenoylcarnitine (C14:1)0.240–0.65
Hexadecanoylcarnitine (C16)15.97 (+)0–7.19
3-OH-hexadecanoylcarnitine (C16-OH)0.120–0.33
Octadecanoylcarnitine (C18)3.53 (+)0–2.52
Octadecenoylcarnitine (C18:1)4.84 (+)0–3.16
3-OH-octadecanoylcarnitine (C18-OH)0.050–0.42

(−): below reference range; (+): above reference range.

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