Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease

To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity w...

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Autores principales: Kodama, Takashi, Togawa, Tadayasu, Tsukimura, Takahiro, Kawashima, Ikuo, Matsuoka, Kazuhiko, Kitakaze, Keisuke, Tsuji, Daisuke, Itoh, Kohji, Ishida, Yo-ichi, Suzuki, Minoru, Suzuki, Toshihiro, Sakuraba, Hitoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3243693/
https://www.ncbi.nlm.nih.gov/pubmed/22205997
http://dx.doi.org/10.1371/journal.pone.0029074
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author Kodama, Takashi
Togawa, Tadayasu
Tsukimura, Takahiro
Kawashima, Ikuo
Matsuoka, Kazuhiko
Kitakaze, Keisuke
Tsuji, Daisuke
Itoh, Kohji
Ishida, Yo-ichi
Suzuki, Minoru
Suzuki, Toshihiro
Sakuraba, Hitoshi
author_facet Kodama, Takashi
Togawa, Tadayasu
Tsukimura, Takahiro
Kawashima, Ikuo
Matsuoka, Kazuhiko
Kitakaze, Keisuke
Tsuji, Daisuke
Itoh, Kohji
Ishida, Yo-ichi
Suzuki, Minoru
Suzuki, Toshihiro
Sakuraba, Hitoshi
author_sort Kodama, Takashi
collection PubMed
description To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.
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spelling pubmed-32436932011-12-28 Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease Kodama, Takashi Togawa, Tadayasu Tsukimura, Takahiro Kawashima, Ikuo Matsuoka, Kazuhiko Kitakaze, Keisuke Tsuji, Daisuke Itoh, Kohji Ishida, Yo-ichi Suzuki, Minoru Suzuki, Toshihiro Sakuraba, Hitoshi PLoS One Research Article To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease. Public Library of Science 2011-12-20 /pmc/articles/PMC3243693/ /pubmed/22205997 http://dx.doi.org/10.1371/journal.pone.0029074 Text en Kodama et al. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
Kodama, Takashi
Togawa, Tadayasu
Tsukimura, Takahiro
Kawashima, Ikuo
Matsuoka, Kazuhiko
Kitakaze, Keisuke
Tsuji, Daisuke
Itoh, Kohji
Ishida, Yo-ichi
Suzuki, Minoru
Suzuki, Toshihiro
Sakuraba, Hitoshi
Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease
title Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease
title_full Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease
title_fullStr Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease
title_full_unstemmed Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease
title_short Lyso-GM2 Ganglioside: A Possible Biomarker of Tay-Sachs Disease and Sandhoff Disease
title_sort lyso-gm2 ganglioside: a possible biomarker of tay-sachs disease and sandhoff disease
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3243693/
https://www.ncbi.nlm.nih.gov/pubmed/22205997
http://dx.doi.org/10.1371/journal.pone.0029074
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