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Hydrogen sulfide (H 2 S) is a gasotransmitter and the smallest signaling thiol metabolite with important roles in human health. The turnover of H 2 S in humans is mainly governed by enzymes of sulfur amino acid metabolism and also by the microbiome. As is the case with other small signaling molecules, disease-promoting effects of H 2 S largely depend on its concentration and compartmentalization. Genetic defects that impair the biogenesis and catabolism of H 2 S have been described; however, a gap in knowledge remains concerning physiological steady-state concentrations of H 2 S and their direct clinical implications. The small size and considerable reactivity of H 2 S renders its quantification in biological samples an experimental challenge. A compilation of methods currently employed to quantify H 2 S in biological specimens is provided in this review. Substantial discrepancy exists in the concentrations of H 2 S determined by different techniques. Available methodologies permit end-point measurement of H 2 S concentration, yet no definitive protocol exists for the continuous, real-time measurement of H 2 S produced by its enzymatic sources. We present a summary of available animal models, monogenic diseases that impair H 2 S metabolism in humans including structure-function relationships of pathogenic mutations, and discuss possible approaches to overcome current limitations of study.

Biosynthesis, Quantification and Genetic Diseases of the Smallest Signaling Thiol Metabolite: Hydrogen Sulfide