[HTML][HTML] Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective

Z Qiu, F Norflus, B Singh, MK Swindell… - Journal of Biological …, 2006 - ASBMB
Interactions between mutant huntingtin (Htt) and a variety of transcription factors including
specificity proteins (Sp) have been suggested as a central mechanism in Huntington
disease (HD). However, the transcriptional activity induced by Htt in neurons that triggers
neuronal death has yet to be fully elucidated. In the current study, we characterized the
relationship of Sp1 to Htt protein aggregation and neuronal cell death. We found increased
levels of Sp1 in neuronal-like PC12 cells expressing mutant Htt, primary striatal neurons …