| Protective effect of sodium butyrate on the cell culture model of Huntington disease |
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| 作者单位： | Zhang Baorong~(1**) Tian Jun~1 Yin Xinzhen~1 Luo Wei~1 Xia Kun~2 (1.Department of Neurology，Second Affiliated Hospital，Zhejiang University School of Medicine，Hangzhou 310009，China; 2.National Laboratory of Medical Genetics of China，Central South University，Changsha 410078，China) |
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| 摘 要： | This study aimed to develop a cell culture model of Huntington disease and observe the effect of sodium butyrate on this cell culture model.Exon 1 of both a wild type and a mutant IT15 gene from the genomic DNA of a healthy adult and a patient with Hunt- ington disease was amplified and cloned into the eukaryotic expression vector pEGFP-C1.Human neuroblastoma SH-SY5Y cells were tran- siently transfected with these recombinant plasmids in the absence and presence of sodium butyrate(0.1,0.2,0.5,1.0 mmol/L).The MTT assay was used to measure cell viability.The results indicated that the N-terminal fragment of mutant huntingtin formed perinuclear and intranuclear aggregates and caused a decrease of SH-SY5Y cell viability.Sodium butyrate inhibited the decrease of SH-SY5Y cell via- bility caused by the N-terminal fragment of mutant huntingtin.This suggests that sodium butyrate has a protective effect on this cell cul- ture model of Huntington disease.
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Protective effect of sodium butyrate on the cell culture model of Huntington disease |
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| Authors: | Zhang Baorong Tian Jun Yin Xinzhen Luo Wei Xia Kun |
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| Abstract: | This study aimed to develop a cell culture model of Huntington disease and observe the effect of sodium butyrate on this cell culture model.Exon 1 of both a wild type and a mutant IT15 gene from the genomic DNA of a healthy adult and a patient with Hunt- ington disease was amplified and cloned into the eukaryotic expression vector pEGFP-C1.Human neuroblastoma SH-SY5Y cells were tran- siently transfected with these recombinant plasmids in the absence and presence of sodium butyrate(0.1,0.2,0.5,1.0 mmol/L).The MTT assay was used to measure cell viability.The results indicated that the N-terminal fragment of mutant huntingtin formed perinuclear and intranuclear aggregates and caused a decrease of SH-SY5Y cell viability.Sodium butyrate inhibited the decrease of SH-SY5Y cell via- bility caused by the N-terminal fragment of mutant huntingtin.This suggests that sodium butyrate has a protective effect on this cell cul- ture model of Huntington disease. |
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| Keywords: | Huntington disease huntingtin sodium butyrate |
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