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

Hdl Handle:
http://hdl.handle.net/10147/267714
Title:
IgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia.
Authors:
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
Affiliation:
University College Dublin, Clinical Research Centre, Mater Misericordiae University Hospital, Ireland.
Citation:
IgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia. 2012, 105 (2):212-20 Mol. Genet. Metab.
Journal:
Molecular genetics and metabolism
Issue Date:
Feb-2012
URI:
http://hdl.handle.net/10147/267714
DOI:
10.1016/j.ymgme.2011.10.018
PubMed ID:
22133299
Additional Links:
http://www.ncbi.nlm.nih.gov/pubmed/22133299
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.
Item Type:
Article
Language:
en
MeSH:
Adult; Biomarkers, Pharmacological; Diet; Drug Tolerance; Female; Galactose; Galactosemias; Glycosylation; Humans; Immunoglobulin G; Male; Polysaccharides
ISSN:
1096-7206

Full metadata record

DC FieldValue Language
dc.contributor.authorCoss, K Pen_GB
dc.contributor.authorByrne, J Cen_GB
dc.contributor.authorComan, D Jen_GB
dc.contributor.authorAdamczyk, Ben_GB
dc.contributor.authorAbrahams, J Len_GB
dc.contributor.authorSaldova, Ren_GB
dc.contributor.authorBrown, A Yen_GB
dc.contributor.authorWalsh, Oen_GB
dc.contributor.authorHendroff, Uen_GB
dc.contributor.authorCarolan, Cen_GB
dc.contributor.authorRudd, P Men_GB
dc.contributor.authorTreacy, E Pen_GB
dc.date.accessioned2013-01-30T15:41:34Z-
dc.date.available2013-01-30T15:41:34Z-
dc.date.issued2012-02-
dc.identifier.citationIgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia. 2012, 105 (2):212-20 Mol. Genet. Metab.en_GB
dc.identifier.issn1096-7206-
dc.identifier.pmid22133299-
dc.identifier.doi10.1016/j.ymgme.2011.10.018-
dc.identifier.urihttp://hdl.handle.net/10147/267714-
dc.description.abstractN-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.en_GB
dc.language.isoenen
dc.relation.urlhttp://www.ncbi.nlm.nih.gov/pubmed/22133299en_GB
dc.rightsArchived with thanks to Molecular genetics and metabolismen_GB
dc.subject.meshAdult-
dc.subject.meshBiomarkers, Pharmacological-
dc.subject.meshDiet-
dc.subject.meshDrug Tolerance-
dc.subject.meshFemale-
dc.subject.meshGalactose-
dc.subject.meshGalactosemias-
dc.subject.meshGlycosylation-
dc.subject.meshHumans-
dc.subject.meshImmunoglobulin G-
dc.subject.meshMale-
dc.subject.meshPolysaccharides-
dc.titleIgG N-glycans as potential biomarkers for determining galactose tolerance in Classical Galactosaemia.en_GB
dc.typeArticleen
dc.contributor.departmentUniversity College Dublin, Clinical Research Centre, Mater Misericordiae University Hospital, Ireland.en_GB
dc.identifier.journalMolecular genetics and metabolismen_GB
dc.description.provinceLeinsteren

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