Hydrogen sulfide production pathway

The hydrogen sulfide production pathway refers to the metabolic routes by which hydrogen sulfide (H₂S) is synthesized endogenously in mammals and other organisms. The main enzymes responsible are cystathionine beta-synthase (CBS), cystathionine gamma-lyase (CSE), and 3-mercaptopyruvate sulfurtransferase (3-MST), with each enzyme exhibiting tissue-specific distribution: CBS is mainly in the brain and liver, CSE in the vasculature and liver, and 3-MST in the brain and vasculature. These enzymes catalyze reactions using amino acid substrates (primarily methionine and cysteine) to generate H₂S, which acts as a signaling molecule influencing processes such as vasodilation, neurotransmission, inflammation modulation, redox signaling, and mitochondrial function. Dysregulation of this pathway is implicated in various diseases, including cardiovascular and neurodegenerative disorders, cancer, and Down syndrome. The pathway is not itself a single drug target but comprises several enzymatic drug targets within sulfur metabolism[1][2][3][5].

Other names

Hydrogen sulfide biosynthesisH2S production pathwayTranssulfuration pathwayH2S generating pathway

Inhibition of hydrogen sulfide-producing enzymes (CBS, CSE, or 3-MST); Exogenous supplementation of H₂S via donor compounds