Huntingtin 150Q Stable PC12 Cell Line

Huntington disease (HD) is an autosomally dominant degenerative disorder resulting from polyglutamine expansion (>37units) in the huntingtin gene. The repeat is often localized in the N-terminal region of huntingtin and the resulting N-terminal fragments accumulate in the nucleus and become toxic to the cells. The HD 150Q protein is predominantly present in the nuclei and as a result, these cells lack normal neurite development. Its control cell line, the Huntingtin 20Q Stable PC12 cell line, in contrast, displays the HD 20Q proteins evenly in the cytoplasm. The two cell lines together provide a valuable model for studies relating to altered gene expression caused by the polyglutamine tracts and the pathology of HD. Generated by the co-transfection of a pCIS expression vector expressing a partial huntingtin cDNA containing 150 CAG repeats and a pCDNA3 expression vector carrying G418 resistance.