ArticleBritish journal of pharmacology2019
Metabolism of sulfur compounds in homocystinurias.
Article in British journal of pharmacology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 40 citations in OpenAlex.
- Homocystinuria: Advances in metabolic and molecular therapies targeting homocysteine pathways (Review).Molecular medicine reports · 2026Review
- Disulfidptosis in tumor progression.Cell death discovery · 2025Review
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- Review
- Synthesis of Sulfides and Persulfides Is Not Impeded by Disruption of Three Canonical Enzymes in Sulfur Metabolism.Antioxidants (Basel, Switzerland) · 2023Article
- Reactive sulfur and selenium species in the regulation of bone homeostasis.Free radical biology & medicine · 2022Review
- Long-term fasting: Multi-system adaptations in humans (GENESIS) study-A single-arm interventional trial.Frontiers in nutrition · 2022Article
- High cysteine diet reduces insulin resistance in SHR-CRP rats.Physiological research · 2021Article
- Reprogrammed transsulfuration promotes basal-like breast tumor progression via realigning cellular cysteine persulfidation.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Hyperhomocysteinemia: Metabolic Role and Animal Studies with a Focus on Cognitive Performance and Decline-A Review.Biomolecules · 2021Review
- Biosynthesis, Quantification and Genetic Diseases of the Smallest Signaling Thiol Metabolite: Hydrogen Sulfide.Antioxidants (Basel, Switzerland) · 2021Review
- Sulfur amino acid restriction, energy metabolism and obesity: a study protocol of an 8-week randomized controlled dietary intervention with whole foods and amino acid supplements.Journal of translational medicine · 2021Article
- Plasma Hydrogen Sulfide Is Positively Associated With Post-operative Survival in Patients Undergoing Surgical Revascularization.Frontiers in cardiovascular medicine · 2021Article
- Sulfur Metabolism Under Stress.Antioxidants & redox signaling · 2020Article
- Analysis of Total Thiols in the Urine of a Cystathionine β-Synthase-Deficient Mouse Model of Homocystinuria Using Hydrophilic Interaction Chromatography.Molecules (Basel, Switzerland) · 2020Article
- Metabolism of sulfur compounds in homocystinurias.British journal of pharmacology · 2019Article
- Highlighted mechanistic aspects in the chemical biology of reactive sulfur species.British journal of pharmacology · 2019Article
- Onco-immunity and therapeutic application of amygdalin: A review.Journal of oral biology and craniofacial researchReview
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeHomocystinurias are rare genetic defects characterized by altered fluxes of sulfur compounds including homocysteine and cysteine. We explored whether the severely perturbed sulfur amino acid metabolism in patients with homocystinurias affects the metabolism of hydrogen sulfide. EXPERIMENTAL APPROACH: We studied 10 treated patients with a block in the conversion of homocysteine to cysteine due to cystathionine β-synthase deficiency (CBSD) and six treated patients with remethylation defects (RMD) and an enhanced flux of sulfur metabolites via transsulfuration. Control groups for CBSD and RMD patients consisted of 22 patients with phenylketonuria on a low-protein diet and of 12 healthy controls respectively. Plasma and urine concentrations of selected sulfur compounds were analysed by HPLC and LC-MS/MS. KEY
resultsPatients with CBSD exhibited plasma concentrations of monobromobimane-detected sulfide similar to appropriate controls. Urinary homolanthionine and thiosulfate in CBSD were increased significantly 1.9 and 3 times suggesting higher hydrogen sulfide synthesis by γ-cystathionase and detoxification respectively. Surprisingly, patients with RMD had significantly lower plasma sulfide levels (53 and 64% of controls) with lower sulfite concentrations, and higher taurine and thiosulfate levels suggesting enhanced cysteine oxidation and hydrogen sulfide catabolism respectively. CONCLUSION AND IMPLICATIONS: The results from this study suggest that severe inherited defects in sulfur amino acid metabolism may be accompanied by only moderately perturbed hydrogen sulfide metabolism and lends support to the hypothesis that enzymes in the transsulfuration pathway may not be the major contributors to the endogenous hydrogen sulfide pool. LINKED ARTICLES: This article is part of a themed section on Chemical Biology of Reactive Sulfur Species. To view the other articles in this section visit http://onlinelibrary.wiley.com/doi/10.1111/bph.v176.4/issuetoc.
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