Rapid Ultraperformance Liquid Chromatography–Tandem Mass Spectrometry Assay for a Characteristic Glycogen-Derived Tetrasaccharide in Pompe Disease and Other Glycogen Storage Diseases | Zendy

Wim J. Sluiter | Zendy; Jeroen C. van den Bosch | Zendy; Daphne A Goudriaan | Zendy; Carin M. Van Gelder | Zendy; Juna M. de Vries | Zendy; J. G. M. Huijmans | Zendy; Arnold Reuser | Zendy; Ans T. van der Ploeg | Zendy; George J. G. Ruijter | Zendy

Open Access

Rapid Ultraperformance Liquid Chromatography–Tandem Mass Spectrometry Assay for a Characteristic Glycogen-Derived Tetrasaccharide in Pompe Disease and Other Glycogen Storage Diseases

Author(s) -

Wim J. Sluiter,

Jeroen C. van den Bosch,

Daphne A Goudriaan,

Carin M. Van Gelder,

Juna M. de Vries,

J. G. M. Huijmans,

Arnold Reuser,

Ans T. van der Ploeg,

George J. G. Ruijter

Publication year - 2012

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/clinchem.2011.178319

Subject(s) - chemistry , chromatography , maltotriose , tandem mass spectrometry , urine , mass spectrometry , maltose , biochemistry , enzyme

Urinary excretion of the tetrasaccharide 6-α-D-glucopyranosyl-maltotriose (Glc₄) is increased in various clinical conditions associated with increased turnover or storage of glycogen, making Glc₄ a potential biomarker for glycogen storage diseases (GSD). We developed an ultraperformance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) assay to detect Glc₄ in urine without interference of the Glc₄ isomer maltotetraose (M₄).

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