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Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment
In mucopolysaccharidoses (MPS), glycosaminoglycans (GAG) accumulate in tissues. In MPS II, approximately two-thirds of patients are cognitively impaired. We investigated levels of GAG in cerebrospinal fluid (CSF) in different populations from four clinical studies (including NCT00920647 and NCT01449...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471392/ https://www.ncbi.nlm.nih.gov/pubmed/28649553 http://dx.doi.org/10.1016/j.ymgmr.2015.11.001 |
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author | Hendriksz, Christian J. Muenzer, Joseph Vanderver, Adeline Davis, Jonathan M. Burton, Barbara K. Mendelsohn, Nancy J. Wang, Nan Pan, Luying Pano, Arian Barbier, Ann J. |
author_facet | Hendriksz, Christian J. Muenzer, Joseph Vanderver, Adeline Davis, Jonathan M. Burton, Barbara K. Mendelsohn, Nancy J. Wang, Nan Pan, Luying Pano, Arian Barbier, Ann J. |
author_sort | Hendriksz, Christian J. |
collection | PubMed |
description | In mucopolysaccharidoses (MPS), glycosaminoglycans (GAG) accumulate in tissues. In MPS II, approximately two-thirds of patients are cognitively impaired. We investigated levels of GAG in cerebrospinal fluid (CSF) in different populations from four clinical studies (including NCT00920647 and NCT01449240). Data indicate that MPS II patients with cognitive impairment have elevated levels of CSF GAG, whereas those with the attenuated phenotype typically have levels falling between those of the cognitively affected patients and healthy controls. |
format | Online Article Text |
id | pubmed-5471392 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-54713922017-06-23 Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment Hendriksz, Christian J. Muenzer, Joseph Vanderver, Adeline Davis, Jonathan M. Burton, Barbara K. Mendelsohn, Nancy J. Wang, Nan Pan, Luying Pano, Arian Barbier, Ann J. Mol Genet Metab Rep Short Communication In mucopolysaccharidoses (MPS), glycosaminoglycans (GAG) accumulate in tissues. In MPS II, approximately two-thirds of patients are cognitively impaired. We investigated levels of GAG in cerebrospinal fluid (CSF) in different populations from four clinical studies (including NCT00920647 and NCT01449240). Data indicate that MPS II patients with cognitive impairment have elevated levels of CSF GAG, whereas those with the attenuated phenotype typically have levels falling between those of the cognitively affected patients and healthy controls. Elsevier 2015-11-09 /pmc/articles/PMC5471392/ /pubmed/28649553 http://dx.doi.org/10.1016/j.ymgmr.2015.11.001 Text en © 2015 The Authors http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Short Communication Hendriksz, Christian J. Muenzer, Joseph Vanderver, Adeline Davis, Jonathan M. Burton, Barbara K. Mendelsohn, Nancy J. Wang, Nan Pan, Luying Pano, Arian Barbier, Ann J. Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment |
title | Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment |
title_full | Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment |
title_fullStr | Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment |
title_full_unstemmed | Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment |
title_short | Levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and Hunter syndrome patients with and without cognitive impairment |
title_sort | levels of glycosaminoglycans in the cerebrospinal fluid of healthy young adults, surrogate-normal children, and hunter syndrome patients with and without cognitive impairment |
topic | Short Communication |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471392/ https://www.ncbi.nlm.nih.gov/pubmed/28649553 http://dx.doi.org/10.1016/j.ymgmr.2015.11.001 |
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