Skip to main content
. Author manuscript; available in PMC: 2015 Mar 4.
Published in final edited form as: Nature. 2014 Mar 26;509(7498):96–100. doi: 10.1038/nature13136

Figure 1. CSE is expressed in the brain and is depleted in Huntington’s disease.

Figure 1

a, CSE expression is detectable in whole-brain lysates. WT, wild type. b, CSE is expressed in different regions of the brain. c, Cse−/− mice show a limb-clasping phenotype. d, Striatal STHdhQ111/Q111 have decreased expression of CSE. e, Quantification of d. n = 3 (means ± s.e.m.); ***P < 0.001. f, Expression of CSE is decreased significantly in all brain regions of 13-week-old R6/2 mice analysed except in the cerebellum. g, Relative quantification of f. n = 6 (means ± s.e.m.) for cortex, striatum and cerebellum; n = 3 (means ± s.e.m.) for other regions; ***P < 0.001; **P < 0.01; NS, not significant. h, Expression of CSE is decreased in striata of Q175 mice. i, Quantification of h. n = 3 (means ± s.e.m.); ***P < 0.001. j, Post-mortem striatal brain samples (P1–P6) from patients with Huntington’s disease show a decrease in CSE expression. k, Relative quantification of j. n = 5 (means ± s.e.m.) for controls; n = 6 (means ± s.e.m.) for samples from patients with Huntington’s disease (HD); ***P < 0.001. l, CSE depletion is increased with the severity of the disease. C1 and C2 are controls;HD1–HD4 are samples from patients with Huntington’s disease. m, Relative quantification of HD grades. n = 3 (means ± s.e.m.) for HD2; n = 4 (means ± s.e.m.) for normal, HD3 and HD4; *P < 0.05 (control versus HD2 and HD2 versus HD3); ***P < 0.001 (control versus HD4). n, CSE expression is decreased in the liver and pancreas. o, Relative quantification of n. n = 3 (means ± s.e.m.); ***P < 0.001.