Cystathionine beta-Synthase
"Cystathionine beta-Synthase" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A multifunctional pyridoxal phosphate enzyme. In the second stage of cysteine biosynthesis it catalyzes the reaction of homocysteine with serine to form cystathionine with the elimination of water. Deficiency of this enzyme leads to HYPERHOMOCYSTEINEMIA and HOMOCYSTINURIA. EC 4.2.1.22.
Descriptor ID |
D003541
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MeSH Number(s) |
D08.811.520.241.300.200
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Concept/Terms |
Cystathionine beta-Synthase- Cystathionine beta-Synthase
- Cystathionine beta Synthase
- beta-Synthase, Cystathionine
- Serine Sulfhydrase
- Sulfhydrase, Serine
- Cystathionine Synthetase
- Synthetase, Cystathionine
- beta-Thionase
- beta Thionase
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Below are MeSH descriptors whose meaning is more general than "Cystathionine beta-Synthase".
Below are MeSH descriptors whose meaning is more specific than "Cystathionine beta-Synthase".
This graph shows the total number of publications written about "Cystathionine beta-Synthase" by people in this website by year, and whether "Cystathionine beta-Synthase" was a major or minor topic of these publications.
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Year | Major Topic | Minor Topic | Total |
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1998 | 1 | 0 | 1 | 2000 | 1 | 0 | 1 | 2002 | 2 | 1 | 3 | 2003 | 1 | 0 | 1 | 2004 | 1 | 0 | 1 | 2006 | 2 | 0 | 2 | 2007 | 1 | 0 | 1 | 2010 | 2 | 0 | 2 | 2011 | 3 | 0 | 3 | 2012 | 1 | 2 | 3 | 2014 | 1 | 0 | 1 | 2015 | 1 | 0 | 1 | 2018 | 0 | 1 | 1 | 2019 | 1 | 0 | 1 | 2020 | 0 | 1 | 1 | 2021 | 2 | 0 | 2 |
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Below are the most recent publications written about "Cystathionine beta-Synthase" by people in Profiles.
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Gupta S, Wang L, Slifker MJ, Cai KQ, Maclean KN, Wasek B, Bottiglieri T, Kruger WD. Analysis of differential neonatal lethality in cystathionine ?-synthase deficient mouse models using metabolic profiling. FASEB J. 2021 06; 35(6):e21629.
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Maclean KN, Jiang H, Phinney WN, Mclagan BM, Roede JR, Stabler SP. Derangement of hepatic polyamine, folate, and methionine cycle metabolism in cystathionine beta-synthase-deficient homocystinuria in the presence and absence of treatment: Possible implications for pathogenesis. Mol Genet Metab. 2021 02; 132(2):128-138.
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Park I, Bublil EM, Glavin F, Majtan T. Interplay of Enzyme Therapy and Dietary Management of Murine Homocystinuria. Nutrients. 2020 Sep 22; 12(9).
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Guerra DD, Bok R, Breen K, Vyas V, Jiang H, MacLean KN, Hurt KJ. Estrogen Regulates Local Cysteine Metabolism in Mouse Myometrium. Reprod Sci. 2021 01; 28(1):79-90.
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Zuhra K, Augsburger F, Majtan T, Szabo C. Cystathionine-?-Synthase: Molecular Regulation and Pharmacological Inhibition. Biomolecules. 2020 04 30; 10(5).
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Bublil EM, Majtan T. Classical homocystinuria: From cystathionine beta-synthase deficiency to novel enzyme therapies. Biochimie. 2020 Jun; 173:48-56.
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Majtan T, Park I, Cox A, Branchford BR, di Paola J, Bublil EM, Kraus JP. Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine-restricted diet or enzyme replacement therapy. FASEB J. 2019 11; 33(11):12477-12486.
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Van Hove JLK, Freehauf CL, Ficicioglu C, Pena LDM, Moreau KL, Henthorn TK, Christians U, Jiang H, Cowan TM, Young SP, Hite M, Friederich MW, Stabler SP, Spector EB, Kronquist KE, Thomas JA, Emmett P, Harrington MJ, Pyle L, Creadon-Swindell G, Wempe MF, MacLean KN. Biomarkers of oxidative stress, inflammation, and vascular dysfunction in inherited cystathionine ?-synthase deficient homocystinuria and the impact of taurine treatment in a phase 1/2 human clinical trial. J Inherit Metab Dis. 2019 05; 42(3):424-437.
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Majtan T, Bublil EM, Park I, Arning E, Bottiglieri T, Glavin F, Kraus JP. Pharmacokinetics and pharmacodynamics of PEGylated truncated human cystathionine beta-synthase for treatment of homocystinuria. Life Sci. 2018 May 01; 200:15-25.
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Maclean KN, Jiang H, Aivazidis S, Kim E, Shearn CT, Harris PS, Petersen DR, Allen RH, Stabler SP, Roede JR. Taurine treatment prevents derangement of the hepatic ?-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: regulatory crosstalk between thiol and sulfinic acid metabolism. FASEB J. 2018 03; 32(3):1265-1280.
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