Measurement of urinary medium chain acyl glycines by gas-chromatography negative-ion chemical ionization mass-spectrometry

S.M.B. Carter, J.M. Midgely, D.G. Watson, R.W. Logan

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is an inborn error of fatty acid metabolism, which is difficult to diagnose, partly because of its unpredictable clinical presentation. A specific diagnostic marker is an increased excretion of certain medium chain acyl glycines. A sensitive and specific method has been developed for the extraction, derivatization, identification and quantitation of urinary medium chain acyl glycines by gas chromatography-negative ion chemical ionization mass spectrometry (GC-NICIMS). The following series of standard acyl glycines has been synthesized and characterized: hexanoyl, octanoyl, 3-phenylpropionyl and suberyl and their respective isotopomers (using13C2-glycine; for use as internal standards). The range of excretion of these compounds in normal subjects has been established using this method and increased excretion of acyl glycines, particularly hexanoyl, 3-phenylpropionyl and suberyl was successfully demonstrated in three MCAD deficient subjects from one family.
LanguageEnglish
Pages969-975
Number of pages6
JournalJournal of Pharmaceutical and Biomedical Analysis
Volume9
Issue number10-12
DOIs
Publication statusPublished - 1991

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Gas chromatography
Gas Chromatography
Glycine
Ionization
Mass spectrometry
Mass Spectrometry
Negative ions
Ions
Acyl-CoA Dehydrogenase
Metabolism
Fatty Acids

Keywords

  • MCAD deficiency
  • acyl glycines
  • hexanoyl glycine
  • octanoyl glycine
  • 3-phenylpropionyl glycine
  • suberyl glycine
  • urine
  • GC—NICIMS

Cite this

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title = "Measurement of urinary medium chain acyl glycines by gas-chromatography negative-ion chemical ionization mass-spectrometry",
abstract = "Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is an inborn error of fatty acid metabolism, which is difficult to diagnose, partly because of its unpredictable clinical presentation. A specific diagnostic marker is an increased excretion of certain medium chain acyl glycines. A sensitive and specific method has been developed for the extraction, derivatization, identification and quantitation of urinary medium chain acyl glycines by gas chromatography-negative ion chemical ionization mass spectrometry (GC-NICIMS). The following series of standard acyl glycines has been synthesized and characterized: hexanoyl, octanoyl, 3-phenylpropionyl and suberyl and their respective isotopomers (using13C2-glycine; for use as internal standards). The range of excretion of these compounds in normal subjects has been established using this method and increased excretion of acyl glycines, particularly hexanoyl, 3-phenylpropionyl and suberyl was successfully demonstrated in three MCAD deficient subjects from one family.",
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author = "S.M.B. Carter and J.M. Midgely and D.G. Watson and R.W. Logan",
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Measurement of urinary medium chain acyl glycines by gas-chromatography negative-ion chemical ionization mass-spectrometry. / Carter, S.M.B.; Midgely, J.M.; Watson, D.G.; Logan, R.W.

In: Journal of Pharmaceutical and Biomedical Analysis, Vol. 9, No. 10-12, 1991, p. 969-975.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Measurement of urinary medium chain acyl glycines by gas-chromatography negative-ion chemical ionization mass-spectrometry

AU - Carter, S.M.B.

AU - Midgely, J.M.

AU - Watson, D.G.

AU - Logan, R.W.

PY - 1991

Y1 - 1991

N2 - Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is an inborn error of fatty acid metabolism, which is difficult to diagnose, partly because of its unpredictable clinical presentation. A specific diagnostic marker is an increased excretion of certain medium chain acyl glycines. A sensitive and specific method has been developed for the extraction, derivatization, identification and quantitation of urinary medium chain acyl glycines by gas chromatography-negative ion chemical ionization mass spectrometry (GC-NICIMS). The following series of standard acyl glycines has been synthesized and characterized: hexanoyl, octanoyl, 3-phenylpropionyl and suberyl and their respective isotopomers (using13C2-glycine; for use as internal standards). The range of excretion of these compounds in normal subjects has been established using this method and increased excretion of acyl glycines, particularly hexanoyl, 3-phenylpropionyl and suberyl was successfully demonstrated in three MCAD deficient subjects from one family.

AB - Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is an inborn error of fatty acid metabolism, which is difficult to diagnose, partly because of its unpredictable clinical presentation. A specific diagnostic marker is an increased excretion of certain medium chain acyl glycines. A sensitive and specific method has been developed for the extraction, derivatization, identification and quantitation of urinary medium chain acyl glycines by gas chromatography-negative ion chemical ionization mass spectrometry (GC-NICIMS). The following series of standard acyl glycines has been synthesized and characterized: hexanoyl, octanoyl, 3-phenylpropionyl and suberyl and their respective isotopomers (using13C2-glycine; for use as internal standards). The range of excretion of these compounds in normal subjects has been established using this method and increased excretion of acyl glycines, particularly hexanoyl, 3-phenylpropionyl and suberyl was successfully demonstrated in three MCAD deficient subjects from one family.

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KW - 3-phenylpropionyl glycine

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