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Tomas Majtan

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Articles 60
Citations 835
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Recent Articles
1.
Zuhra K, Petrosino M, Janickova L, Petric J, Ascencao K, Vignane T, et al.
Nat Metab . 2025 Mar; PMID: 40033006
Small, gaseous molecules such as nitric oxide, carbon monoxide and hydrogen sulfide are produced as signalling molecules in mammalian cells. Here, we show that low concentrations of cyanide are generated...
2.
Lee H, Gupta K, Wang L, Dunbrack R, Majtan T, Kruger W
Protein Sci . 2024 Nov; 33(12):e5223. PMID: 39548832
Cystathionine beta-synthase (CBS) is an evolutionarily conserved enzyme that plays a key role in mammalian sulfur amino acid biochemistry, mutations in which are the cause of classical homocystinuria (HCU), an...
3.
Philipp T, Bottiglieri T, Clapper W, Liu K, Rodems S, Szabo C, et al.
Redox Biol . 2024 Oct; 77:103383. PMID: 39366068
Homocystinuria (HCU) due to cystathionine beta-synthase (CBS) deficiency is characterized by elevated plasma and tissue homocysteine levels. There is no cure, but HCU is typically managed by methionine/protein restriction and...
4.
Mijatovic E, Ascencao K, Szabo C, Majtan T
Protein Sci . 2024 Jul; 33(8):e5123. PMID: 39041895
Homocystinuria (HCU) due to cystathionine beta-synthase (CBS) deficiency is the most common inborn error of sulfur amino acid metabolism. Recent work suggests that missense pathogenic mutations-regardless of their topology-cause instability...
5.
Kozich V, Majtan T
J Inherit Metab Dis . 2024 Jun; 47(5):841-859. PMID: 38873792
This review summarises progress in the research of homocystinuria (HCU) in the past three decades. HCU due to cystathionine β-synthase (CBS) was discovered in 1962, and Prof. Jan Peter Kraus...
6.
Majtan T, Olsen T, Sokolova J, Krijt J, Krizkova M, Ida T, et al.
Redox Biol . 2024 Jun; 73:103222. PMID: 38843767
Background: Cystathionine β-synthase (CBS)-deficient homocystinuria (HCU) is an inherited disorder of sulfur amino acid metabolism with varying severity and organ complications, and a limited knowledge about underlying pathophysiological processes. Here...
7.
Pedretti M, Fernandez-Rodriguez C, Conter C, Oyenarte I, Favretto F, Di Matteo A, et al.
Sci Rep . 2024 Apr; 14(1):9364. PMID: 38654065
The escalating drug resistance among microorganisms underscores the urgent need for innovative therapeutic strategies and a comprehensive understanding of bacteria's defense mechanisms against oxidative stress and antibiotics. Among the recently...
8.
Collard R, Majtan T
Mol Cell Biol . 2023 Dec; 43(12):664-674. PMID: 38051092
Homocystinuria (HCU), an inherited metabolic disorder caused by lack of cystathionine beta-synthase (CBS) activity, is chiefly caused by misfolding of single amino acid residue missense pathogenic variants. Previous studies showed...
9.
Majtan T, Kozich V, Kruger W
Br J Pharmacol . 2022 Nov; 180(3):264-278. PMID: 36417581
Cystathionine beta-synthase (CBS)-deficient homocystinuria (HCU) is the most common inborn error of sulfur amino acid metabolism. The pyridoxine non-responsive form of the disease manifests itself by massively increasing plasma and...
10.
Zuhra K, Petrosino M, Gupta B, Panagaki T, Cecconi M, Myrianthopoulos V, et al.
Nitric Oxide . 2022 Aug; 128:12-24. PMID: 35973674
Epigallocatechin gallate (EGCG) is the main bioactive component of green tea. Through screening of a small library of natural compounds, we discovered that EGCG inhibits cystathionine β-synthase (CBS), a major...