Enzymatic activities of cystathionine γ-lyase (CSE) and cystathionine β-synthase (CBS) in the Q175 model of Huntington’s disease. (A–C) Enzymatic activity of CSE at 2, 6, and 12 months in the cortex, cerebellum, hippocampus, and striatum in Q175 mice and their wild-type counterparts. H2S production from 10 mM L-cysteine in the presence of the cofactor, pyridoxal 5-phosphate (PLP at 250 mM), using the methylene blue assay (D–F). Enzymatic activity of CBS at 2, 6, and 12 months in the cortex, cerebellum, hippocampus, and striatum in Q175 mice and their wild-type counterparts. Activity was measured using the substrates for CBS (15 mM L-homocysteine and 10 mM L-cysteine) using the methylene blue assay. n = 3, SEM. *** p < 0.001.