IgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia

Coss, K.P., Byrne, J.C., Coman, D.J., Adamczyk, B., Abrahams, J.L., Saldova, R., Brown, A.Y., Walsh, O., Hendroff, U., Carolan, C., Rudd, P.M. and Treacy, E.P. (2012) IgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia. Molecular Genetics and Metabolism, 105 2: 1-9. doi:10.1016/j.ymgme.2011.10.018

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Author Coss, K.P.
Byrne, J.C.
Coman, D.J.
Adamczyk, B.
Abrahams, J.L.
Saldova, R.
Brown, A.Y.
Walsh, O.
Hendroff, U.
Carolan, C.
Rudd, P.M.
Treacy, E.P.
Title IgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia
Journal name Molecular Genetics and Metabolism   Check publisher's open access policy
ISSN 1096-7192
Publication date 2012-02
Year available 2011
Sub-type Article (original research)
DOI 10.1016/j.ymgme.2011.10.018
Volume 105
Issue 2
Start page 1
End page 9
Total pages 9
Place of publication Maryland Heights, MO, United States
Publisher Academic Press
Collection year 2012
Language eng
Abstract N-glycan processing and assembly defects have been demonstrated in untreated and partially treated patients with Classical Galactosaemia. These defects may contribute to the ongoing pathophysiology of this disease. The aim of this study was to develop an informative method of studying differential galactose tolerance levels and diet control in individuals with Galactosaemia, compared to the standard biochemical markers. Ten Galactosaemia adults with normal intellectual outcomes were analyzed in the study. Five subjects followed galactose liberalization, increments of 300. mg to 4000. mg/day over 16. weeks, and were compared to five adult Galactosaemia controls on a galactose restricted diet. All study subjects underwent clinical and biochemical monitoring of red blood cell galactose-1-phosphate (RBC Gal-1-P) and urinary galactitol levels. Serum N-glycans were isolated and analyzed by normal phase high-performance liquid chromatography (NP-HPLC) with galactosylation of IgG used as a specific biomarker of galactose tolerance. IgG N-glycan profiles showed consistent individual alterations in response to diet liberalization. The individual profiles were improved for all, but one study subject, at a galactose intake of 1000. mg/day, with decreases in agalactosylated (G0) and increases in digalactosylated (G2) N-glycans. We conclude that IgG N-glycan profiling is an improved method of monitoring variable galactosylation and determining individual galactose tolerance in Galactosaemia compared to the standard methods.
Keyword Biomarkers
Diet liberalization
IgG N-glycans
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ
Additional Notes Available online 6 November 2011.

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2012 Collection
School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 18 times in Thomson Reuters Web of Science Article | Citations
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Created: Mon, 19 Dec 2011, 11:33:33 EST by Matthew Lamb on behalf of School of Medicine