The Journal of pharmacy and pharmacology · 2015
Effects of cyclodextrins on GM1-gangliosides in fibroblasts from GM1-gangliosidosis patients.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Maeda Yuki, Motoyama Keiichi, Higashi Taishi, Horikoshi Yuka, Takeo Toru, Nakagata Naomi, Kurauchi Yuki, Katsuki Hiroshi, Ishitsuka Yoichi, Kondo Yuki, Irie Tetsumi, Furuya Hirokazu, Era Takumi, Arima Hidetoshi
Аннотация
OBJECTIVES: GM1-gangliosidosis is an inherited disorder characterized by the accumulation of GM1-gangliosides in many tissues and organs, particularly in the brain. Currently, there is no treatment available for patients with ganglioside storage diseases. Therefore, we investigated the effects of cyclodextrins (CyDs) on the GM1-ganglioside level in EA1 cells, fibroblasts from patients with GM1-gangliosidosis.
METHODS: The concentrations of cholesterol and phospholipids in supernatants were determined by Cholesterol E-test Wako and Phospholipid C-test Wako, respectively. The effects of CyDs on GM1-ganglioside levels in EA1 cells using fluorescence-labelled cholera toxin B-subunit, which can bind to GM1-gangliosides specifically, were investigated by flow cytometry and confocal laser scanning microscopy.
KEY FINDINGS: The treatment with methylated CyDs, hydroxypropylated CyDs and branched CyDs decreased GM1-ganglioside levels in EA1 cells at 1 mm for 24 h. Unexpectedly, there was no significant change in the efflux of cholesterol or phospholipids from the cells after treatment with CyDs under the same experimental conditions, indicating that the efflux of membrane components is not associated with down-regulation of GM1-ganglioside levels in EA1 cells upon CyDs treatment.
CONCLUSIONS: CyDs may have the potential as drugs for GM1-gangliosidosis, although the mechanism should be thereafter clarified.
METHODS: The concentrations of cholesterol and phospholipids in supernatants were determined by Cholesterol E-test Wako and Phospholipid C-test Wako, respectively. The effects of CyDs on GM1-ganglioside levels in EA1 cells using fluorescence-labelled cholera toxin B-subunit, which can bind to GM1-gangliosides specifically, were investigated by flow cytometry and confocal laser scanning microscopy.
KEY FINDINGS: The treatment with methylated CyDs, hydroxypropylated CyDs and branched CyDs decreased GM1-ganglioside levels in EA1 cells at 1 mm for 24 h. Unexpectedly, there was no significant change in the efflux of cholesterol or phospholipids from the cells after treatment with CyDs under the same experimental conditions, indicating that the efflux of membrane components is not associated with down-regulation of GM1-ganglioside levels in EA1 cells upon CyDs treatment.
CONCLUSIONS: CyDs may have the potential as drugs for GM1-gangliosidosis, although the mechanism should be thereafter clarified.