Biosynthesis, Quantification and Genetic Diseases of the Smallest Signaling Thiol Metabolite: Hydrogen Sulfide
Hydrogen sulfide (H<sub>2</sub>S) is a gasotransmitter and the smallest signaling thiol metabolite with important roles in human health. The turnover of H<sub>2</sub>S in humans is mainly governed by enzymes of sulfur amino acid metabolism and also by the microbiome. As is th...
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| Main Authors: | , , , , |
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| Format: | Book |
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MDPI AG,
2021-07-01T00:00:00Z.
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| Summary: | Hydrogen sulfide (H<sub>2</sub>S) is a gasotransmitter and the smallest signaling thiol metabolite with important roles in human health. The turnover of H<sub>2</sub>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<sub>2</sub>S largely depend on its concentration and compartmentalization. Genetic defects that impair the biogenesis and catabolism of H<sub>2</sub>S have been described; however, a gap in knowledge remains concerning physiological steady-state concentrations of H<sub>2</sub>S and their direct clinical implications. The small size and considerable reactivity of H<sub>2</sub>S renders its quantification in biological samples an experimental challenge. A compilation of methods currently employed to quantify H<sub>2</sub>S in biological specimens is provided in this review. Substantial discrepancy exists in the concentrations of H<sub>2</sub>S determined by different techniques. Available methodologies permit end-point measurement of H<sub>2</sub>S concentration, yet no definitive protocol exists for the continuous, real-time measurement of H<sub>2</sub>S produced by its enzymatic sources. We present a summary of available animal models, monogenic diseases that impair H<sub>2</sub>S metabolism in humans including structure-function relationships of pathogenic mutations, and discuss possible approaches to overcome current limitations of study. |
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| Item Description: | 10.3390/antiox10071065 2076-3921 |