cystathionine β-synthase
Enzyme replacement with PEGylated cystathionine β synthase ameliorates homocystinuria in murine model
... from cystathionine β-synthase (CBS) deficiency, is the most common defect of sulfur amino acid ...to cystathionine that is then converted to ...of cystathionine and ...
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Severe hyperhomocysteinemia due to cystathionine β-synthase deficiency, and Factor V Leiden mutation in a patient with recurrent venous thrombosis
... We performed a skin biopsy and cultured skin fibro- blasts to examine known inborn error of cobalamin me- tabolism. The incorporation of both [ 14 C]-propionate and [ 14 C]-methyltetrahydrofolate and the uptake of [ 57 ...
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5-FU targets rpL3 to induce mitochondrial apoptosis via cystathionine-β-synthase in colon cancer cells lacking p53
... Recent findings revealed in cancer cells novel stress response pathways, which in response to many chemotherapeutic drugs causing nucleolar stress, will function independently from tumor protein p53 (p53) and still lead ...
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Affinity of Cystathionine β Synthase for Pyridoxal 5′ Phosphate in Cultured Cells: A MECHANISM FOR PYRIDOXINE RESPONSIVE HOMOCYSTINURIA
... Previous attempts to correlate in vivo pyridoxine-responsiveness with in vitro assays of cystathionine b-synthase activity in synthase-deficient homocystinuric patients have been only partially ...
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Betaine reduces β-amyloid-induced paralysis through activation of cystathionine-β-synthase in an Alzheimer model of Caenorhabditis elegans
... RNA interference (RNAi) was performed by using the feeding method. As described elsewhere, it was con- ducted in liquid cultures, which were enriched with RNAi bacteria [16, 17]. In brief, in the RNAse III- deficient E. ...
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A critical role for cystathionine β synthase in hydrogen sulfide mediated hypoxic relaxation of the coronary artery
... Krebs–Henseleit buffer (mM): NaCl 118, KCl 4.8, CaCl 2 ·H 2 O 1.3, NaHCO 3 25.0, KH 2 PO 4 1.2, MgSO 4 ·1.2 and glucose 11.1 (Fisher, Loughborough). U46619 (Tocris Bioscience, Bristol) was dissolved in ethanol at 10 mM, ...
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Cystathionine-β-Synthase Inhibition for Colon Cancer: Enhancement of the Efficacy of Aminooxyacetic Acid via the Prodrug Approach
... enzyme, cystathionine γ-lyase (8); this additional pharmacological action may also contribute to the observed changes in transsulfuration pathway intermedi- ...
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Homocystinuria: EVIDENCE FOR THREE DISTINCT CLASSES OF CYSTATHIONINE β SYNTHASE MUTANTS IN CULTURED FIBROBLASTS
... cystathionine b-synthase activity with added pyridoxal-5¢-phosphate; in the other four, the cofactor addition increased activity only modestly, as in controls. Two of seven lines from nonresponsive patients ...
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Coronary artery hypoxic vasorelaxation is augmented by perivascular adipose tissue through a mechanism involving hydrogen sulfide and cystathionine β synthase
... 5% (v/v) β-mercaptoethanol, 2.5% (v/v) bromophenol blue, 1.5 M Tris–HCl, pH 6.8), were heated at 95 °C for 5 min. Subsequently, electrophoresis was carried out on 4–20% Tris- glycine (PAGE) Gold Precast Gels ...
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Interactions among methylenetetrahydrofolate reductase (MTHFR) and cystathionine β-synthase (CBS) polymorphisms - a cross-sectional study: multiple heterozygosis as a risk factor for higher homocysteine levels and vaso-occlusive episodes.
... CBS is the first key enzyme of the homocysteine trans-sulfuration pathway; it catalyzes the condensation of Hcy with serine to form cystathionine and, ultimately, cysteine (Song et al., 2006; Miranda-Vilela, ...
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CBS domains form energy sensing modules whose binding of adenosine ligands is disrupted by disease mutations
... DNA cloning and expression. PCDNA plasmids encoding rat γ1 (29) and human γ2 and γ3 (28) were used as tem- plates for PCR. DNAs encoding all four CBS domains (CBS1-4) from γ1 (residues 42–324), γ2 (residues 274–569), and ...
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Transsulfuration is an active pathway for cysteine biosynthesis in Trypanosoma rangeli
... of cystathionine β-synthase (CβS) and cysteine synthase (CS) from bacteria, yeast, plants, and parasitic protozoa as queries to identify putative ...
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Emerging pharmacological tools to control hydrogen sulfide signaling in critical illness
... CBS: Cystathionine β -synthase; CNS: Central nervous system; CO: Carbon monoxide; CoQ: Coenzyme Q; CSE: Cystathionine γ -lyase; ETC: Electron transport chain; ETHE1: Ethylmalonic ...
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Long noncoding RNAs in DNA methylation: new players stepping into the old game
... a total of 338 genomic loci, of which 161 peaks are near 150 annotated genes. Notably, 31 of these sites overlapped with differentially methylated regions (DMRs) previously found in colon cancer samples versus matched ...
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Cardiovascular disease and resuscitated septic shock lead to the downregulation of the H2S-producing enzyme cystathionine-γ-lyase in the porcine coronary artery
... 3-MST: 3-Mercaptopyruvate sulfurtransferase; ADRP: Adipocyte differentiation-related protein; CA: Coronary artery; CAD: Coronary artery disease; CBS: Cystathionine- β -synthase; CO: Carb[r] ...
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Modulation of hydrogen sulfide by vascular hypoxia
... It has long been known that hydrogen sulfide (H 2 S) is endogenously produced by multiple enzymes, including cystathionine β -synthase (CBS), cystathionine γ -lyase (CSE), and ...
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Association of neural tube defects with maternal alterations and genetic polymorphisms in one-carbon metabolic pathway
... Moreover, a few studies have suggested impaired homocysteine (Hcy) metabolism might be involved in the protective mechanism of folate [8]. Several studies have also revealed that mothers having children with NTDs possess ...
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Homocystinuria due to cystathionine synthase deficiency Studies of nitrogen balance and sulfur excretion
... seven cystathionine synthase-deficient patients, and a single cystathioninuric patient on semisynthetic diets containing low-adequate amounts of methionine and very low amounts of methionine and very low ...
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Original Article Increased nicotinamide phosphoribosyltransferase and cystathionine-beta-synthase in oral cavity squamous cell carcinomas
... malignancies. Cystathionine-beta-synthase synthesizes hydrogen sulfide and shows increased expression in several malignan- ...and cystathionine-beta-synthase protein levels in oral squamous ...
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Induced pluripotent stem cells in Alzheimer’s disease: applications for disease modeling and cell-replacement therapy
... The identification of AD causal genes resulted in the generation of more than 150 transgenic AD mouse models, by over-expressing one or more mutated genes such as human APP, PSEN1/2, or Tau [24]. These trans- genic ...
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