Mass spectrometric identification of 2-hydroxydodecanedioic acid and its homologues in urine from patients with hopantenate therapy during clinical episode.

  title={Mass spectrometric identification of 2-hydroxydodecanedioic acid and its homologues in urine from patients with hopantenate therapy during clinical episode.},
  author={Masahiro Matsumoto and Tomiko Kuhara and Yoshito Inoue and Toshihiro Shinka and Isamu Matsumoto and M Kajita},
  journal={Biomedical \& environmental mass spectrometry},
  volume={19 3},
Urine from patients with calcium-4-(2,4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate (hopantenate) therapy during episodes of Reye's-like syndrome was found to contain a number of unusual dicarboxylic acids in high concentrations; odd- and even-numbered medium-chain dicarboxylic acids, alpha-hydroxydicarboxylic acids and beta-hydroxydicarboxylic acids. The abnormal excretion of dicarboxylic acids, alpha- and beta-hydroxydicarboxylic acids disappeared after discontinuance of… 
Stereoselective analysis of 2-hydroxysebacic acid in urine of patients with Zellweger syndrome and of premature infants fed withmedium-chain triglycerides
Using enantioselective multidimensional gas chromatography—mass spectrometry, an increased excretion of 2R-HS was observed in all investigated ZS patients and 2- HS was also present in urine samples of premature infants fed a medium-chain triglyceride (MCT)-containing diet.
A study on α-ketoadipic aciduria by gas chromatographic-mass spectrometry
Following up two cases of α-ketoadipic aciduria clinically and metabolically using organic solvent extraction, new urease-pretreatment and gas chromatography-mass spectrometry (GC/MS) showed heterogeneity, and the clinical manifestations of this metabolic disorder showed heterogeneity.
Gas chromatographic/mass spectrometric identification of 3-hydroxydicarboxylic acids in urine.
The identification of the urinary, saturated and unsaturated 3-hydroxydicarboxylic acids as their trimethylsilyl derivatives was performed with electron impact and positive chemical ionization gas chromatography/mass spectrometry.
Erythematous skin lesions and partial deficiency of 3-methylcrotonyl-CoA carboxylase in peripheral lymphocytes in a patient with propionic acidaemia
A patient with propionic acidaemia who exhibited erythematous skin lesions and a moderately reduced activity of 3methylcrotonyl-CoA carboxylase (MCC, EC in peripheral lymphocytes is described.
Changes in urinary level and configuration ratio of D-lactic acid in patients with short bowel syndrome.
  • Y. Inoue, T. Shinka, T. Kuhara
  • Medicine
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences
  • 2007
Synthesis Characterization and Biological Activity Study of New Schiff and Mannich Bases and Some Metal Complexes Derived from Isatin and Dithiooxamide
Two new Schiff and Mannich bases were prepared from condensation reaction of new Schiff base 3-(1′-N-dithiooxamide)iminoisatin (SBH) with morpholine or diphenylamine respectively in presence of formaldehyde and their structures were characterized by IR, 1HNMR, mass spectrometry, and CHN analyses.
An asymptomatic infant with isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency detected by newborn screening for maple syrup urine disease
An asymptomatic male infant with isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency who came to medical attention by newborn mass screening due to elevated blood leucine is described.
Electron ionization mass spectra of indolenines obtained using sector and ion trap mass spectrometers.
The electron impact (EI)-induced fragmentations of 18 indolenines were studied using both double-focusing and ion trap mass spectrometers to provide correlations of characteristic fragment ions with specific structural differences.


3-hydroxydecanedioic acid and related homologues: urinary metabolites in ketoacidosis.
It is suggested that the 3-hydroxy dicarboxylic acids are formed from fatty acids by a combination of omega-oxidation and incomplete beta-Oxidation, both of which are found in ketoacidosis.
Mass spectrometric identification of 2-hydroxy-sebacic acid in the urines of patients with neonatal adrenoleukodystrophy and Zellweger syndrome.
The urines of children with neonatal adrenoleukodystrophy and Zellweger syndrome contained an excess of unusual even- and odd-numbered dicarboxylic acids with a chain length of from 5 to 15 carbon atoms, as well as 2-hydroxy-compounds, which appears as an additional useful marker of these peroxisomal disorders.
Mass spectrometric identification of abnormal aromatic compounds in the urine of a child with Reye's like syndrome.
The urine of a young child with hypoglycemia and a Reye's like syndrome contained an excess of unusual aromatic products with a three carbon chain, phenylpropionylglycine and 3- and 4-hydroxyphenyl)propionic acids, as well as of organic acids usually found in fatty acid beta-oxidation defects.
Mass spectrometry in diagnosis of metabolic disorders.
Capillary gas chromatography/mass spectrometry with computerized library search is used primarily to separate and identify urinary organic acids and is suggested to be an important diagnostic tool for many years ahead.
Effect of γ-Aminobutyric Acid Derivatives, Especially, Homopantothenic Acid, on Excitability of the Brain
Various derivatives of γ-aminobutyric acid (GABA) including homopantothenic acid (HOPA) were prepared and their seizureand excitation-suppressing action and sleep-prolonging effect were examined. The
Electron impact‐induced rearrangement of trimethylsilyl groups in long chain compounds
Mass spectra of trimethylsilyl (TMS) derivatives of long chain dicarboxylic acids, hydroxy acids, cyano acids, and terminal diols, dithiols and diamines have been examined. A number of fragmentation
Urinary organic acid profiles of Reye's syndrome patients.