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Cystathionine β-synthase (human)
1987Publisher Summary Cystathionine β-synthase (L-serine hydro-lyase [adding homocysteine]) is a pyridoxal 5’-phosphate-dependent enzyme in the transsuifuration pathway of higher eukaryotes which catalyzes the condensation of serine and homocysteine to form cystathionine.
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Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria
2017Classical homocystinuria (HCU) is the most common loss-of-function inbornerror of sulfur amino acid metabolism. HCU is caused by a deficiency in enzymaticdegradation of homocysteine, a toxic intermediate of methionine transformationto cysteine, chiefly due to missense mutations in the cystathionine betasynthase(CBS) gene.
Majtan, Tomas +6 more
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Marine natural products as inhibitors of cystathionine beta-synthase activity
Bioorganic & Medicinal Chemistry Letters, 2015A library consisting of characterized marine natural products as well as synthetic derivatives was screened for compounds capable of inhibiting the production of hydrogen sulfide (H2S) by cystathionine beta-synthase (CBS). Eight hits were validated and shown to inhibit CBS activity with IC50 values ranging from 83 to 187μM.
Megan K, Thorson +6 more
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Biogenesis of Hydrogen Sulfide and Thioethers by Cystathionine Beta-Synthase
Antioxidants & Redox Signaling, 2018Aims: The transsulfuration pathway enzymes cystathionine beta-synthase (CBS) and cystathionine gammalyaseare thought to be the major source of hydrogen sulfide (H2S). In this study, we assessed the role of CBS inH2S biogenesis.Results: We show that despite discouraging enzyme kinetics of alternative H2S-producing reactions utilizingcysteine compared ...
Majtan, Tomas +8 more
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The natural history of homocystinuria due to cystathionine beta-synthase deficiency.
American journal of human genetics, 1985An international questionnaire survey has been conducted to define better the natural history of homocystinuria due to cystathionine beta-synthase deficiency and permit evaluation of treatment. Data were compiled for 629 patients. Among patients not discovered by newborn screening, B6-responsive individuals on the average have significantly better ...
Mudd SH +9 more
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Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway
Molecular Genetics and Metabolism, 2007Cystathionine beta synthase (CBS) deficiency is a metabolic disorder that is biochemically characterized by severe hyperhomocysteinemia. In order to show the effects of CBS deficiency onto the activity of the enzymes involved in the remethylation pathway, we used the well characterized genetic model of severe hyperhomocysteinemia in mice.
Jm, Alberto +7 more
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Cystathionine beta-synthase and gamma-cystathionase in helminths.
Parasitology research, 1992The activities of gamma-cystathionase and cystathionine beta-synthase were investigated in a range of gastrointestinal, free-living and entomophagous nematodes. Although nematode gamma-cystathionase used the same range of substrates as the mammalian hepatic enzyme, its activity was extremely low and there were significant interspecies variations with ...
J, Walker, J, Barrett
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Postoperative thromboemboli in cystathionine beta-synthase deficiency
American Heart Journal, 1984G M, Jackson +4 more
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Management of pregnancy in cystathionine beta-synthase deficiency
Molecular Genetics and Metabolism, 2022K Arduini, L Hitchins, D Bartke
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