Figure 2.

Expression of B3galt4 (GM1/GD1b synthase) is downregulated in striatal cell models of HD and in fibroblasts from HD patients. A, B, Gene expression analysis by quantitative PCR in striatal knock-in cells, rat striatal parental (ST14A) and mHtt-expressing cells (N548-120Q) (A), and human fibroblasts (B). Data were normalized to cyclophilin A expression. HD fibroblasts were compared with age-matched controls at the same passage number (C1 and C2). Subscripts represent the number of CAG repeats in the HD gene of the affected individuals. Additional information on the human fibroblast lines is provided in supplemental Table 1 (available at www.jneurosci.org as supplemental material). Data are the mean ± SD of two to four independent experiments. 7/7, STHdh^7/7; 111/111, STHdh^111/111; *p < 0.05; **p < 0.001; ***p < 0.0001.