Lyso-GM2 ganglioside: A possible biomarker of Tay-Sachs disease and sandhoff disease

Takashi Kodama; Tadayasu Togawa; Takahiro Tsukimura; Ikuo Kawashima; Kazuhiko Matsuoka; Keisuke Kitakaze; Daisuke Tsuji; Kohji Itoh; Yo ichi Ishida; Minoru Suzuki; Toshihiro Suzuki; Hitoshi Sakuraba

doi:10.1371/journal.pone.0029074

Lyso-GM2 ganglioside: A possible biomarker of Tay-Sachs disease and sandhoff disease

Takashi Kodama
, Tadayasu Togawa
, Takahiro Tsukimura
, Ikuo Kawashima
, Kazuhiko Matsuoka
, Keisuke Kitakaze
, Daisuke Tsuji
, Kohji Itoh
, Yo ichi Ishida
, Minoru Suzuki
, Toshihiro Suzuki
, Hitoshi Sakuraba

Research output: Contribution to journal › Article › peer-review

40 Scopus citations

Abstract

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.

Original language	English
Article number	e29074
Journal	PLoS ONE
Volume	6
Issue number	12
DOIs	https://doi.org/10.1371/journal.pone.0029074
State	Published - 20 Nov 2011
Externally published	Yes

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title = "Lyso-GM2 ganglioside: A possible biomarker of Tay-Sachs disease and sandhoff disease",

abstract = "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.",

author = "Takashi Kodama and Tadayasu Togawa and Takahiro Tsukimura and Ikuo Kawashima and Kazuhiko Matsuoka and Keisuke Kitakaze and Daisuke Tsuji and Kohji Itoh and Ishida, \{Yo ichi\} and Minoru Suzuki and Toshihiro Suzuki and Hitoshi Sakuraba",

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doi = "10.1371/journal.pone.0029074",

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AU - Kodama, Takashi

AU - Togawa, Tadayasu

AU - Tsukimura, Takahiro

AU - Kawashima, Ikuo

AU - Matsuoka, Kazuhiko

AU - Kitakaze, Keisuke

AU - Tsuji, Daisuke

AU - Itoh, Kohji

AU - Ishida, Yo ichi

AU - Suzuki, Minoru

AU - Suzuki, Toshihiro

AU - Sakuraba, Hitoshi

PY - 2011/11/20

Y1 - 2011/11/20

N2 - 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.

AB - 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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ER -

Lyso-GM2 ganglioside: A possible biomarker of Tay-Sachs disease and sandhoff disease

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