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A modified liquid chromatography/tandem mass spectrometry method for predominant disaccharide units of urinary glycosaminoglycans in patients with mucopolysaccharidoses

Overview of attention for article published in Orphanet Journal of Rare Diseases, September 2014
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Title
A modified liquid chromatography/tandem mass spectrometry method for predominant disaccharide units of urinary glycosaminoglycans in patients with mucopolysaccharidoses
Published in
Orphanet Journal of Rare Diseases, September 2014
DOI 10.1186/s13023-014-0135-3
Pubmed ID
Authors

Chih-Kuang Chuang, Hsiang-Yu Lin, Tuen-Jen Wang, Chia-Chen Tsai, Hsuan-Liang Liu, Shuan-Pei Lin

Abstract

BackgroundThe identification of acid mucopolysaccharide by the liquid chromatography/tandem mass spectrometry method (LC-MS/MS) of the predominant disaccharide units of glycosaminoglycans (GAGs) (chondroitin sulfate, CS; dermatan sulfate, DS; heparan sulfate, HS) after methanolysis is validated and applicable for mucopolysaccharidosis (MPS) type determination.MethodsA total of 76 urine samples were collected and analyzed, from nine MPS I patients, 13 MPS II patients, seven MPS III patients, eight MPS VI patients, and 39 normal controls. Urinary GAG was first precipitated by the Alcian blue method followed by a treatment of 3 N HCl methanol. The protonated species of the methylated disaccharide products were detected by using a multiple reaction monitoring experiment. Internal standards, [2H6] CS, [2H6] DS and [2H6] HS, were prepared in-house by deuteriomethanolysis of CS, DS and HS.ResultsOne particular disaccharide for each GAG was selected, in which the parent ion and its daughter ion after collision were m/z 426.1¿¿¿236.2 for DS (m/z 432¿¿¿239 for dimers derived from [2H6] CS and [2H6] DS) and m/z 384.2¿¿¿161.9 for HS (m/z 390.4¿¿¿162.5 for the [2H6] HS dimer). The quantities of DS and HS were determined, which varied from one MPS type to the other. The results can be used to evaluate the severity of MPS subgroups, as well as urinary GAG amelioration at follow-up after enzyme replacement therapy (ERT).ConclusionsThe modified LC¿MS/MS method for MPS type determination is specific, sensitive, validated, accurate, and applicable for simultaneous quantifications of urinary DS and HS. This method can help to make correct diagnosis of MPS patients and evaluate the effectiveness of ERT.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 44 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 44 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 8 18%
Student > Doctoral Student 6 14%
Student > Master 6 14%
Researcher 5 11%
Professor > Associate Professor 3 7%
Other 7 16%
Unknown 9 20%
Readers by discipline Count As %
Medicine and Dentistry 10 23%
Chemistry 9 20%
Biochemistry, Genetics and Molecular Biology 6 14%
Agricultural and Biological Sciences 4 9%
Psychology 2 5%
Other 5 11%
Unknown 8 18%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 4. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 11 May 2022.
All research outputs
#6,406,754
of 22,763,032 outputs
Outputs from Orphanet Journal of Rare Diseases
#870
of 2,611 outputs
Outputs of similar age
#63,424
of 237,378 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#21
of 44 outputs
Altmetric has tracked 22,763,032 research outputs across all sources so far. This one has received more attention than most of these and is in the 70th percentile.
So far Altmetric has tracked 2,611 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. This one has gotten more attention than average, scoring higher than 64% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 237,378 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 72% of its contemporaries.
We're also able to compare this research output to 44 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 50% of its contemporaries.