Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency

Yasuhiro Maeda, Tetsuya Ito, Hironori Ohmi, Kyoko Yokoi, Yoko Nakajima, Akihito Ueta, Yukihisa Kurono, Hajime Togari, Naruji Sugiyama

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of organic acidemias in newborns. However, C5OH-I has not been used as a standard in tandem mass spectrometric (MS/MS) assays because its isolation is difficult. We developed a new synthesis of C5OH-I and investigated its behavior by MS/MS. A method using the multiple reaction monitoring (MRM) mode of MS/MS with HPLC was developed which provides high accuracy, precision and reproducibility. Acylcarnitine profiles in the serum and urine of a patient with multiple carboxylase deficiency (MCD) showed increased levels compared to a healthy patient.

Original languageEnglish
Pages (from-to)154-159
Number of pages6
JournalJournal of Chromatography B: Analytical Technologies in the Biomedical and Life Sciences
Volume870
Issue number2
DOIs
Publication statusPublished - 15-07-2008
Externally publishedYes

Fingerprint

Multiple Carboxylase Deficiency
Liquid chromatography
Tandem Mass Spectrometry
Liquid Chromatography
Mass spectrometry
Assays
Blood
Urine
Monitoring
Serum
High Pressure Liquid Chromatography
Newborn Infant
acylcarnitine
3-hydroxyisovalerylcarnitine

All Science Journal Classification (ASJC) codes

  • Analytical Chemistry
  • Biochemistry
  • Clinical Biochemistry
  • Cell Biology

Cite this

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title = "Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency",
abstract = "Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of organic acidemias in newborns. However, C5OH-I has not been used as a standard in tandem mass spectrometric (MS/MS) assays because its isolation is difficult. We developed a new synthesis of C5OH-I and investigated its behavior by MS/MS. A method using the multiple reaction monitoring (MRM) mode of MS/MS with HPLC was developed which provides high accuracy, precision and reproducibility. Acylcarnitine profiles in the serum and urine of a patient with multiple carboxylase deficiency (MCD) showed increased levels compared to a healthy patient.",
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Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency. / Maeda, Yasuhiro; Ito, Tetsuya; Ohmi, Hironori; Yokoi, Kyoko; Nakajima, Yoko; Ueta, Akihito; Kurono, Yukihisa; Togari, Hajime; Sugiyama, Naruji.

In: Journal of Chromatography B: Analytical Technologies in the Biomedical and Life Sciences, Vol. 870, No. 2, 15.07.2008, p. 154-159.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency

AU - Maeda, Yasuhiro

AU - Ito, Tetsuya

AU - Ohmi, Hironori

AU - Yokoi, Kyoko

AU - Nakajima, Yoko

AU - Ueta, Akihito

AU - Kurono, Yukihisa

AU - Togari, Hajime

AU - Sugiyama, Naruji

PY - 2008/7/15

Y1 - 2008/7/15

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