Cargando…
Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment
In Alpha-mannosidosis (MIM 248500) the patients accumulate mainly unbranched oligosaccharide chains in the lysosomes in all body tissues, including the brain. With ensuing therapeutic modalities in man (BMT and ERT) non-invasive methods of monitoring the effect of treatment are needed. Paramount is...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Netherlands
2011
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3173639/ https://www.ncbi.nlm.nih.gov/pubmed/21541723 http://dx.doi.org/10.1007/s10545-011-9331-7 |
_version_ | 1782211980922191872 |
---|---|
author | Avenarius, Derk Frederik Matthaus Svendsen, John-Sigurd Malm, Dag |
author_facet | Avenarius, Derk Frederik Matthaus Svendsen, John-Sigurd Malm, Dag |
author_sort | Avenarius, Derk Frederik Matthaus |
collection | PubMed |
description | In Alpha-mannosidosis (MIM 248500) the patients accumulate mainly unbranched oligosaccharide chains in the lysosomes in all body tissues, including the brain. With ensuing therapeutic modalities in man (BMT and ERT) non-invasive methods of monitoring the effect of treatment are needed. Paramount is the possible effect of the treatment on the brain, since this organ is regarded as difficult to reach because of the blood-brain barrier. We therefore performed proton nuclear magnetic resonance spectroscopy (MRS) of the brain in two untreated patients, and a 16-year-old patient treated with BMT at the age of 10 to assess whether this non-invasive method could be applied in the monitoring of the accumulation of abnormal chemicals in the brain of patients. We found an abnormal peak that was not present in the treated patient. A similar pattern was also found in MRS of urine from patients, reflecting the concentration of oligosaccharides in serum and tissues. We therefore conclude that MRS can be a useful method to monitor the effect of treatment for Alpha-Mannosidosis. |
format | Online Article Text |
id | pubmed-3173639 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Springer Netherlands |
record_format | MEDLINE/PubMed |
spelling | pubmed-31736392011-09-26 Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment Avenarius, Derk Frederik Matthaus Svendsen, John-Sigurd Malm, Dag J Inherit Metab Dis Original Article In Alpha-mannosidosis (MIM 248500) the patients accumulate mainly unbranched oligosaccharide chains in the lysosomes in all body tissues, including the brain. With ensuing therapeutic modalities in man (BMT and ERT) non-invasive methods of monitoring the effect of treatment are needed. Paramount is the possible effect of the treatment on the brain, since this organ is regarded as difficult to reach because of the blood-brain barrier. We therefore performed proton nuclear magnetic resonance spectroscopy (MRS) of the brain in two untreated patients, and a 16-year-old patient treated with BMT at the age of 10 to assess whether this non-invasive method could be applied in the monitoring of the accumulation of abnormal chemicals in the brain of patients. We found an abnormal peak that was not present in the treated patient. A similar pattern was also found in MRS of urine from patients, reflecting the concentration of oligosaccharides in serum and tissues. We therefore conclude that MRS can be a useful method to monitor the effect of treatment for Alpha-Mannosidosis. Springer Netherlands 2011-05-04 2011 /pmc/articles/PMC3173639/ /pubmed/21541723 http://dx.doi.org/10.1007/s10545-011-9331-7 Text en © The Author(s) 2011 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited. |
spellingShingle | Original Article Avenarius, Derk Frederik Matthaus Svendsen, John-Sigurd Malm, Dag Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
title | Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
title_full | Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
title_fullStr | Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
title_full_unstemmed | Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
title_short | Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
title_sort | proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3173639/ https://www.ncbi.nlm.nih.gov/pubmed/21541723 http://dx.doi.org/10.1007/s10545-011-9331-7 |
work_keys_str_mv | AT avenariusderkfrederikmatthaus protonnuclearmagneticresonancespectroscopicdetectionofoligomannosidicnglycansinalphamannosidosisamethodofmonitoringtreatment AT svendsenjohnsigurd protonnuclearmagneticresonancespectroscopicdetectionofoligomannosidicnglycansinalphamannosidosisamethodofmonitoringtreatment AT malmdag protonnuclearmagneticresonancespectroscopicdetectionofoligomannosidicnglycansinalphamannosidosisamethodofmonitoringtreatment |