Literature DB >> 10070613

Identification of new medium-chain acylcarnitines present in urine of a patient with medium-chain acyl-CoA dehydrogenase deficiency.

R Libert1, F Van Hoof, M Thillaye, M F Vincent, M C Nassogne, V Stroobant, E de Hoffmann, A Schanck.   

Abstract

Previously undescribed medium-chain acylcarnitines were identified in a urine sample from a patient with medium-chain acyl-CoA dehydrogenase deficiency. These are the 4-methylvaleryl, 4- and 5-methylhexanoyl, 6-methylheptanoyl-, 6-methyloctanoyl-, 4,5-dimethylhexanoyl- and 4,7-decadienoylcarnitines. Their chemical structures were obtained by gas chromatographymass spectrometry analysis of their fatty acid moieties as picolinyl esters.

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Year:  1999        PMID： 10070613     DOI： 10.1023/a:1005482828392

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  7 in total

1.  Simple and unambiguous method for identifying urinary acylcarnitines using gas chromatography--mass spectrometry.

Authors:  S Lowes; M E Rose
Journal:  Analyst       Date:  1990-05       Impact factor: 4.616

2.  The detection of abnormal metabolites in MCAD deficiency: a new method.

Authors:  W Blom; A C Polder-Mol; H H Kelholt-Dijkman; L Hierck; J G Huijmans
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

3.  Identification of new medium-chain acylcarnitines present in normal human urine.

Authors:  R Libert; F Van Hoof; M Thillaye; M F Vincent; M C Nassogne; V Stroobant; E de Hoffmann; A Schanck
Journal:  Anal Biochem       Date:  1997-09-05       Impact factor: 3.365

4.  Medium-chain acyl-CoA dehydrogenase deficiency. Diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine.

Authors:  P Rinaldo; J J O'Shea; P M Coates; D E Hale; C A Stanley; K Tanaka
Journal:  N Engl J Med       Date:  1988-11-17       Impact factor: 91.245

5.  Octanoylglucuronide excretion in patients with a defective oxidation of medium-chain fatty acids.

Authors:  M Duran; D Ketting; R van Vossen; T E Beckeringh; L Dorland; L Bruinvis; S K Wadman
Journal:  Clin Chim Acta       Date:  1985-11-15       Impact factor: 3.786

6.  Identification and quantification of intermediates of unsaturated fatty acid metabolism in plasma of patients with fatty acid oxidation disorders.

Authors:  W Onkenhout; V Venizelos; P F van der Poel; M P van den Heuvel; B J Poorthuis
Journal:  Clin Chem       Date:  1995-10       Impact factor: 8.327

7.  Mass spectra of the picolinyl esters of isomeric mono- and dienoic fatty acids.

Authors:  W W Christie; E Y Brechany; R T Holman
Journal:  Lipids       Date:  1987-04       Impact factor: 1.880
  7 in total
  3 in total

1.  Metabolism of propionic acid to a novel acyl-coenzyme A thioester by mammalian cell lines and platelets.

Authors:  Nathaniel W Snyder; Sankha S Basu; Andrew J Worth; Clementina Mesaros; Ian A Blair
Journal:  J Lipid Res       Date:  2014-11-25       Impact factor: 5.922

2.  Picolinyl ester fragmentation mechanism studies with application to the identification of acylcarnitine acyl groups following transesterification.

Authors:  Shuming Yang; Paul Minkler; Charles Hoppel; Kou-Yi Tserng
Journal:  J Am Soc Mass Spectrom       Date:  2006-08-09       Impact factor: 3.109

3.  Pseudo-glutarylcarnitinaemia in medium-chain acyl-CoA dehydrogenase deficiency detected by tandem mass spectrometry newborn screening.

Authors:  N Napolitano; V Wiley; J J Pitt
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982
  3 in total

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