US EPA

1462631 

Journal Article 

Protective effect of sodium butyrate on the cell culture model of Huntington disease 

Zhang, B; Jun, T; Yin, X; Wei, Luo; Kun, Xia 

2007 

1 

Progress in Natural Science
ISSN: 1002-0071 

17 

7 

784-788 

English 

WOS:000249234100004 

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 Huntington disease was amplified and cloned into the eukaryotic expression vector pEGFP-C1. Human neuroblastoma SH-SY5Y cells were transiently 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 viability caused by the N-terminal fragment of mutant huntingtin. This suggests that sodium butyrate has a protective effect on this cell culture model of Huntington disease. 

Huntington disease; huntingtin; sodium butyrate