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Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease.

Publication ,  Journal Article
An, Y; Young, SP; Hillman, SL; Van Hove, JL; Chen, YT; Millington, DS
Published in: Anal Biochem
December 1, 2000

A HPLC method associated with butyl-p-aminobenzoate derivatization has been developed for the analysis of a tetraglucose oligomer, Glcalpha1-6Glcalpha1-4Glcalpha1-4Glc, designated Glc(4), in biological fluids. This tetraglucose, normally excreted in the urine, has previously been shown to be elevated in a number of pathological conditions including Pompe disease (glycogen storage disease type II), which is caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase. Concentrations of Glc(4) in both urine and plasma were established for the age ranges of <1, 1-5, 6-10, 11-20, and >20 years, both in normal individuals and in a cohort of 21 patients with enzymatically confirmed Pompe disease. The Glc(4) concentration decreased with age in both groups, but all the patients had elevated Glc(4) levels compared with age-matched controls. Electrospray tandem mass spectrometry was employed to establish the homogeneity of the HPLC peak for Glc(4) and to investigate the identity of other unusual oligosaccharides excreted in patient urine. Our results demonstrate that this method is suitable for application in clinical laboratories to help establish the diagnosis of Pompe disease.

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Published In

Anal Biochem

DOI

ISSN

0003-2697

Publication Date

December 1, 2000

Volume

287

Issue

1

Start / End Page

136 / 143

Location

United States

Related Subject Headings

  • Sensitivity and Specificity
  • Oligosaccharides
  • Middle Aged
  • Male
  • Infant
  • Humans
  • Glycogen Storage Disease Type II
  • Female
  • Chromatography, Liquid
  • Chromatography, High Pressure Liquid
 

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An, Y., Young, S. P., Hillman, S. L., Van Hove, J. L., Chen, Y. T., & Millington, D. S. (2000). Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease. Anal Biochem, 287(1), 136–143. https://doi.org/10.1006/abio.2000.4838
An, Y., S. P. Young, S. L. Hillman, J. L. Van Hove, Y. T. Chen, and D. S. Millington. “Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease.Anal Biochem 287, no. 1 (December 1, 2000): 136–43. https://doi.org/10.1006/abio.2000.4838.
An Y, Young SP, Hillman SL, Van Hove JL, Chen YT, Millington DS. Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease. Anal Biochem. 2000 Dec 1;287(1):136–43.
An, Y., et al. “Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease.Anal Biochem, vol. 287, no. 1, Dec. 2000, pp. 136–43. Pubmed, doi:10.1006/abio.2000.4838.
An Y, Young SP, Hillman SL, Van Hove JL, Chen YT, Millington DS. Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease. Anal Biochem. 2000 Dec 1;287(1):136–143.
Journal cover image

Published In

Anal Biochem

DOI

ISSN

0003-2697

Publication Date

December 1, 2000

Volume

287

Issue

1

Start / End Page

136 / 143

Location

United States

Related Subject Headings

  • Sensitivity and Specificity
  • Oligosaccharides
  • Middle Aged
  • Male
  • Infant
  • Humans
  • Glycogen Storage Disease Type II
  • Female
  • Chromatography, Liquid
  • Chromatography, High Pressure Liquid