Linked Life Data

11078593

Source:http://linkedlifedata.com/resource/pubmed/id/11078593

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
2000-12-13
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Dec
pubmed:issn
0003-2697
pubmed:author
pubmed:copyrightInfo
Copyright 2000 Academic Press.
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
287
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
136-43
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
2000
pubmed:articleTitle
Liquid chromatographic assay for a glucose tetrasaccharide, a putative biomarker for the diagnosis of Pompe disease.
pubmed:affiliation
Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, 99 TW Alexander Drive, Research Triangle Park, North Carolina 27709, USA.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't