Results 31 to 40 of about 975,983 (211)

Screening of Heteroaromatic Scaffolds against Cystathionine Beta-Synthase Enables Identification of Substituted Pyrazolo[3,4-c]Pyridines as Potent and Selective Orthosteric Inhibitors. [PDF]

Molecules, 2020
Cystathionine β-synthase (CBS) is a key enzyme in the production of the signaling molecule hydrogen sulfide, deregulation of which is known to contribute to a range of serious pathological states.
Fantel AM, Myrianthopoulos V, Georgoulis A, Lougiakis N, Zantza I, Lamprinidis G, Augsburger F, Marakos P, Vorgias CE, Szabo C, Pouli N, Papapetropoulos A, Mikros E.   +12 more
europepmc   +2 more sources

Cystathionine beta synthase deficiency and brain edema associated with methionine excess under betaine supplementation: Four new cases and a review of the evidence. [PDF]

JIMD Rep, 2020
CBS deficient individuals undergoing betaine supplementation without sufficient dietary methionine restriction can develop severe hypermethioninemia and brain edema. Brain edema has also been observed in individuals with severe hypermethioninemia without
Schwahn BC, Scheffner T, Stepman H, Verloo P, Das AM, Fletcher J, Blom HJ, Benoist JF, Barshop BA, Barea JJ, Feigenbaum A.   +10 more
europepmc   +2 more sources

Cystathionine beta synthase expression in mouse retina. [PDF]

Curr Eye Res, 2013
Purpose: Cystathionine β-synthase (CBS), a key enzyme in the transsulfuration metabolic pathway, converts homocysteine to cystathionine, which is converted to cysteine required for the synthesis of major retinal antioxidant glutathione (GSH).
Markand S, Tawfik A, Ha Y, Gnana-Prakasam J, Sonne S, Ganapathy V, Sen N, Xian M, Smith SB.   +8 more
europepmc   +6 more sources

Pathogenic Homocystinuria-Associated T236N Mutation Dramatically Alters the Biochemical Properties of Cystathionine Beta-Synthase Protein. [PDF]

Biomedicines
Background: Cystathione beta-synthase (CBS) T236N is a novel mutation associated with pyridoxine non-responsiveness, which presents a significant difficulty in the medical treatment of homocystinuria. Reported severe phenotypes in homocystinuria patients
Al-Sadeq DW, Thanassoulas A, Theodoridou M, Nasrallah GK, Nomikos M.   +4 more
europepmc   +2 more sources

Identification and Functional Analysis of Cystathionine Beta-Synthase Gene Mutations in Chinese Families with Classical Homocystinuria. [PDF]

Biomedicines
Background: Homocystinuria caused by cystathionine β-synthase (CBS) deficiency is the most common congenital disorder related to sulfur amino acid metabolism, manifested by neurological, vascular, and connective tissue involvement.
Liu X, Liu X, Liu J, Guo J, Nie D, Wang J.   +5 more
europepmc   +2 more sources

Differential expression of cystathionine beta synthase in adolescents with Down syndrome: impact on adiposity. [PDF]

J Diabetes Metab Disord, 2022
Meguid NA, Hemimi M, Ghozlan SAS, Kandeel WA, Hashish AF, Gouda AS, Nazim WS, Mohamed MF.   +7 more
europepmc   +2 more sources

Homocystinuria due to cystathionine beta-synthase (CBS) deficiency in Russia: Molecular and clinical characterization. [PDF]

Mol Genet Metab Rep, 2018
We present the results of the 45-year clinical observation of 27 Russian homocystinuria patients. We made a mutation analysis of the CBS gene for thirteen patients from eleven unrelated genealogies.
Voskoboeva E, Semyachkina A, Yablonskaya M, Nikolaeva E.   +3 more
europepmc   +2 more sources

Cystathionine beta-synthase promotes anoikis resistance and transcoelomic metastasis in ovarian cancer via SP1- ITGB1 regulation. [PDF]

Exp Hematol Oncol
Anoikis resistance is crucial for ovarian cancer (OvCa) transcoelomic metastasis, during which exfoliated OvCa cells survive as spheroids before invading the omentum.
Shaw P, Dey Bhowmik A, Jaswal AP, Velayutham R, Chiliveru S, Ricketts S, Xu C, Dhanasekaran DN, Zhao ZJ, Bhattacharya R, Mukherjee P, Dwivedi SKD, Rao G.   +12 more
europepmc   +2 more sources

Asymmetric dimethylarginine in adults with cystathionine beta-synthase deficiency.

Atherosclerosis, 2012
Item does not contain fulltextIn hyperhomocysteinemia (HHcy), an independent risk factor for cardiovascular diseases, endothelial dysfunction due to reduced bioavailability of nitric oxide is a consistent finding.
Rivera, I., Rocha, M.S., Tavares de Almeida, I., Kluijtmans, L.A.J., Janssen, M.C.H., Teerlink, T., Castro, R., Jakobs, C., Blom, H.J., Smulders, Y.   +9 more
core   +6 more sources

Biogenesis of Hydrogen Sulfide and Thioethers by Cystathionine Beta-Synthase

Antioxidants & Redox Signaling, 2018
Aims: The transsulfuration pathway enzymes cystathionine beta-synthase (CBS) and cystathionine gamma-lyase are thought to be the major source of hydrogen sulfide (H2S). In this study, we assessed the role of CBS in H2S biogenesis.
T. Majtan, J. Krijt, J. Sokolová, Michaela Křížková, Maria A. Ralat, J. Kent, J. Gregory, V. Kožich, J. Kraus   +8 more
semanticscholar   +3 more sources