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JIMD Reports, Volume 21

Overview of attention for book
Cover of 'JIMD Reports, Volume 21'

Table of Contents

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    Book Overview
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    Chapter 330 Di-sulfated Keratan Sulfate as a Novel Biomarker for Mucopolysaccharidosis II, IVA, and IVB.
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    Chapter 347 Metabolic Clinic Atlas: Organization of Care for Children with Inherited Metabolic Disease in Canada
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    Chapter 365 Maternal Phenylketonuria: Long-term Outcomes in Offspring and Post-pregnancy Maternal Characteristics.
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    Chapter 373 Assessing Psychological Functioning in Metabolic Disorders: Validation of the Adaptive Behavior Assessment System, Second Edition (ABAS-II), and the Behavior Rating Inventory of Executive Function (BRIEF) for Identification of Individuals at Risk
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    Chapter 377 Glutaric Acidemia Type 1-Clinico-Molecular Profile and Novel Mutations in GCDH Gene in Indian Patients
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    Chapter 384 A Retrospective Survey Studying the Impact of Fabry Disease on Pregnancy
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    Chapter 387 Evaluation of Implementation, Adaptation and Use of the Recently Proposed Urea Cycle Disorders Guidelines
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    Chapter 389 Autophagy in Natural History and After ERT in Glycogenosis Type II
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    Chapter 393 Is l -Carnitine Supplementation Beneficial in 3-Methylcrotonyl-CoA Carboxylase Deficiency?
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    Chapter 394 Neurodevelopmental and Cognitive Outcomes of Classical Homocystinuria: Experience from Qatar.
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    Chapter 399 Diet History Is a Reliable Predictor of Suboptimal Docosahexaenoic Acid Levels in Adult Patients with Phenylketonuria
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    Chapter 401 Successful Pregnancy in a Woman with Maple Syrup Urine Disease: Case Report
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    Chapter 402 Hepatic Copper Accumulation: A Novel Feature in Transient Infantile Liver Failure Due to TRMU Mutations?
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    Chapter 404 Infantile Cases of Sitosterolaemia with Novel Mutations in the ABCG5 Gene: Extreme Hypercholesterolaemia is Exacerbated by Breastfeeding
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    Chapter 441 Molecular Characterization of QDPR Gene in Iranian Families with BH4 Deficiency: Reporting Novel and Recurrent Mutations
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    Chapter 448 Erratum to: Glutaric Acidemia Type 1-Clinico-Molecular Profile and Novel Mutations in GCDH Gene in Indian Patients
Attention for Chapter 330: Di-sulfated Keratan Sulfate as a Novel Biomarker for Mucopolysaccharidosis II, IVA, and IVB.
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  • High Attention Score compared to outputs of the same age and source (83rd percentile)

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Chapter title
Di-sulfated Keratan Sulfate as a Novel Biomarker for Mucopolysaccharidosis II, IVA, and IVB.
Chapter number 330
Book title
JIMD Reports, Volume 21
Published in
JIMD Reports, February 2015
DOI 10.1007/8904_2014_330
Pubmed ID
25712379
Book ISBNs
978-3-66-247171-5, 978-3-66-247172-2
Authors

Tsutomu Shimada, Shunji Tomatsu, Robert W Mason, Eriko Yasuda, William G Mackenzie, Jobayer Hossain, Yuniko Shibata, Adriana M Montaño, Francyne Kubaski, Roberto Giugliani, Seiji Yamaguchi, Yasuyuki Suzuki, Kenji E Orii, Toshiyuki Fukao, Tadao Orii, Robert W. Mason, William G. Mackenzie, Adriana M. Montaño, Kenji E. Orii, Shimada, Tsutomu, Tomatsu, Shunji, Mason, Robert W., Yasuda, Eriko, Mackenzie, William G., Hossain, Jobayer, Shibata, Yuniko, Montaño, Adriana M., Kubaski, Francyne, Giugliani, Roberto, Yamaguchi, Seiji, Suzuki, Yasuyuki, Orii, Kenji E., Fukao, Toshiyuki, Orii, Tadao

Abstract

Keratan sulfate (KS) is a storage material in mucopolysaccharidosis IV (MPS IV). However, no detailed analysis has been reported on subclasses of KS: mono-sulfated KS and di-sulfated KS. We established a novel method to distinguish and quantify mono- and di-sulfated KS using liquid chromatography-tandem mass spectrometry and measured both KS levels in various specimens.Di-sulfated KS was dominant in shark cartilage and rat serum, while mono-sulfated KS was dominant in bovine cornea and human serum. Levels of both mono- and di-sulfated KS varied with age in the blood and urine from control subjects and patients with MPS II and IVA. The mean levels of both forms of KS in the plasma/serum from patients with MPS II, IVA, and IVB were elevated compared with that in age-matched controls. Di-sulfated KS provided more significant difference between MPS IVA and the age-matched controls than mono-sulfated KS. The ratio of di-sulfated KS to total KS in plasma/serum increased with age in control subjects and patients with MPS II but was age independent in MPS IVA patients. Consequently, this ratio can discriminate younger MPS IVA patients from controls. Levels of mono- and di-sulfated KS in urine of MPS IVA and IVB patients were all higher than age-matched controls for all ages studied.In conclusion, the level of di-sulfated KS and its ratio to total KS can distinguish control subjects from patients with MPS II, IVA, and IVB, indicating that di-sulfated KS may be a novel biomarker for these disorders.

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X Demographics

The data shown below were collected from the profile of 1 X user who shared this research output. Click here to find out more about how the information was compiled.
 Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 24 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 24 100%

Demographic breakdown

Readers by professional status Count As %
Student > Master 3 13%
Student > Doctoral Student 3 13%
Professor 2 8%
Researcher 2 8%
Student > Ph. D. Student 2 8%
Other 4 17%
Unknown 8 33%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 5 21%
Medicine and Dentistry 4 17%
Nursing and Health Professions 2 8%
Chemistry 2 8%
Immunology and Microbiology 1 4%
Other 2 8%
Unknown 8 33%
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 03 January 2016.
All research outputs
#6,415,132
of 22,793,427 outputs
Outputs from JIMD Reports
#116
of 544 outputs
Outputs of similar age
#73,164
of 255,481 outputs
Outputs of similar age from JIMD Reports
#3
of 18 outputs
Altmetric has tracked 22,793,427 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 544 research outputs from this source. They receive a mean Attention Score of 2.8. This one has done well, scoring higher than 78% 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 255,481 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 70% of its contemporaries.
We're also able to compare this research output to 18 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 83% of its contemporaries.

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