Profiling of Pentose Phosphate Pathway Intermediates in Blood Spots by Tandem Mass Spectrometry: Application to Transaldolase Deficiency | Zendy

Jojanneke H.J. Huck | Zendy; Eduard A. Struys | Zendy; Nanda M. Verhoeven | Zendy; Cornelis Jakobs | Zendy; Marjo S. van der Knaap | Zendy

Open Access

Profiling of Pentose Phosphate Pathway Intermediates in Blood Spots by Tandem Mass Spectrometry: Application to Transaldolase Deficiency

Author(s) -

Jojanneke H.J. Huck,

Eduard A. Struys,

Nanda M. Verhoeven,

Cornelis Jakobs,

Marjo S. van der Knaap

Publication year - 2003

Publication title -

clinical chemistry

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.705

H-Index - 218

eISSN - 1530-8561

pISSN - 0009-9147

DOI - 10.1373/49.8.1375

Subject(s) - transaldolase , sugar phosphates , pentose phosphate pathway , pentose , chemistry , tandem mass spectrometry , chromatography , hexose , phosphate , biochemistry , mass spectrometry , liquid chromatography–mass spectrometry , metabolism , glycolysis , enzyme , fermentation

Recently, several patients with abnormal polyol profiles in body fluids have been reported, but the origins of these polyols are unknown. We hypothesized that they are derived from sugar phosphate intermediates of the pentose phosphate pathway (PPP), and we developed a semiquantitative method for profiling of pentose phosphate pathway intermediates.

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