Figure 1.
Expression of cystathionine γ-lyase (CSE) in the Q175 model of Huntington’s disease. (A) The transsulfuration pathway via which cysteine and hydrogen sulfide (H2S) is generated. Transsulfuration refers to the transfer of sulfur from homocysteine (which is derived from dietary methionine) to cysteine. Cystathionine β-synthase (CBS) condenses serine with homocysteine to generate cystathionine, which is acted on by CSE to produce cysteine. Cysteine and homocysteine serve as substrates of CSE or CBS to generate H2S. (B) Expression of CSE at 2, 6, and 12 months in the striatum, the region most profoundly affected in HD, in Q175 mice and their wild-type counterparts. (C) Quantification of B. (D–F) Expression of CSE at 2, 6, and 12 months in the cortex, hippocampus and cerebellum. n = 3, SEM. *** p < 0.001.