Zobrazeno 1 - 10
of 80
pro vyhledávání: '"Jakub Krijt"'
Autor:
Tomas Majtan, Thomas Olsen, Jitka Sokolova, Jakub Krijt, Michaela Křížková, Tomoaki Ida, Tamás Ditrói, Hana Hansikova, Ondrej Vit, Jiri Petrak, Ladislav Kuchař, Warren D. Kruger, Péter Nagy, Takaaki Akaike, Viktor Kožich
Publikováno v:
Redox Biology, Vol 73, Iss , Pp 103222- (2024)
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.
Externí odkaz:
https://doaj.org/article/b8708cd31b8443ea933830c25a0f91db
Autor:
Thomas Olsen, Kathrine J. Vinknes, Kristýna Barvíková, Emma Stolt, Sindre Lee-Ødegård, Hannibal Troensegaard, Hanna Johannessen, Amany Elshorbagy, Jitka Sokolová, Jakub Krijt, Michaela Křížková, Tamás Ditrói, Péter Nagy, Bente Øvrebø, Helga Refsum, Magne Thoresen, Kjetil Retterstøl, Viktor Kožich
Publikováno v:
Redox Biology, Vol 73, Iss , Pp 103192- (2024)
Background: In animals, dietary sulfur amino acid restriction (SAAR) improves metabolic health, possibly mediated by altering sulfur amino acid metabolism and enhanced anti-obesogenic processes in adipose tissue. Aim: To assess the effects of SAAR ov
Externí odkaz:
https://doaj.org/article/25c59b32605a4c42bd104f1491f8959c
Autor:
Viktor Kožich, Bernd C Schwahn, Jitka Sokolová, Michaela Křížková, Tamas Ditroi, Jakub Krijt, Youssef Khalil, Tomáš Křížek, Tereza Vaculíková-Fantlová, Blanka Stibůrková, Philippa Mills, Peter Clayton, Kristýna Barvíková, Holger Blessing, Jolanta Sykut-Cegielska, Carlo Dionisi-Vici, Serena Gasperini, Ángeles García-Cazorla, Tobias B Haack, Tomáš Honzík, Pavel Ješina, Alice Kuster, Lucia Laugwitz, Diego Martinelli, Francesco Porta, René Santer, Guenter Schwarz, Peter Nagy
Publikováno v:
Redox Biology, Vol 58, Iss , Pp 102517- (2022)
Regulation of H2S homeostasis in humans is poorly understood. Therefore, we assessed the importance of individual enzymes in synthesis and catabolism of H2S by studying patients with respective genetic defects. We analyzed sulfur compounds (including
Externí odkaz:
https://doaj.org/article/c104284c656d4baba6f0fc3dc95350b4
Autor:
Anna Koslová, Pavel Trefil, Jitka Mucksová, Veronika Krchlíková, Jiří Plachý, Jakub Krijt, Markéta Reinišová, Dana Kučerová, Josef Geryk, Jiří Kalina, Filip Šenigl, Daniel Elleder, Viktor Kožich, Jiří Hejnar
Publikováno v:
Viruses, Vol 13, Iss 12, p 2504 (2021)
The chicken Tva cell surface protein, a member of the low-density lipoprotein receptor family, has been identified as an entry receptor for avian leukosis virus of classic subgroup A and newly emerging subgroup K. Because both viruses represent an im
Externí odkaz:
https://doaj.org/article/dfebb64862e64b58b3293fd1b2c3fcfe
Publikováno v:
Human Mutation. 41:1662-1670
Classical homocystinuria (HCU) is an inborn error of metabolism caused by loss of cystathionine β-synthase (CBS) activity with the concomitant buildup of homocysteine. In knockout (KO) mice, a mouse model of HCU, complete lack of CBS is neonatally l
Autor:
Anna Klimovskaia, Beat Thöny, Ralph Fingerhut, Jakub Krijt, Jean-Marc Nuoffer, Gabriella Allegri, Hiu Man Grisch-Chan, Viktor Kožich, Benjamin Causton, Tanja Scherer, Johannes Häberle, Sereina Deplazes, Carmen Diez-Fernandez, Nicole Rimann, Jonathan W. Leff
Publikováno v:
Journal of Inherited Metabolic Disease. 42:1064-1076
The most common ureagenesis defect is X-linked ornithine transcarbamylase (OTC) deficiency which is a main target for novel therapeutic interventions. The spf ash mouse model carries a variant (c.386G>A, p.Arg129His) that is also found in patients. M
Autor:
Jitka Sokolová, Viktor Kožich, Jakub Krijt, Tamás Ditrói, Péter Nagy, Michaela Křížková, Pavel Ješina
Publikováno v:
British Journal of Pharmacology. 176:594-606
Background and purpose Homocystinurias are rare genetic defects characterized by altered fluxes of sulfur compounds including homocysteine and cysteine. We explored whether the severely perturbed sulfur amino acid metabolism in patients with homocyst
Publikováno v:
Nucleosides, Nucleotides and Nucleic Acids. 37:324-328
Hereditary xanthinuria (type I) is caused by an inherited deficiency of the xanthine oxidorectase (XDH/XO), and is characterized by very low concentration of uric acid in blood and urine and high concentration of urinary xanthine, leading to urolithi
Autor:
Tomas Majtan, Erez M. Bublil, Insun Park, Jan P. Kraus, Viktor Kožich, Helena Hůlková, Jakub Krijt
Classical homocystinuria (HCU) is an inborn error of sulfur amino acid metabolism caused by deficient activity of cystathionine β-synthase (CBS), resulting in an accumulation of homocysteine and a concomitant decrease of cystathionine and cysteine i
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ea841549e33c4713f7ec2bf7c5446868
Autor:
Jitka Sokolová, Roman Vozdek, Viktor Kožich, Jakub Krijt, Petra Melenovská, Tomas Majtan, Jan P. Kraus, Pavel Ješina
Publikováno v:
Biochimie. 126:14-20
Two enzymes in the transsulfuration pathway of homocysteine -cystathionine beta-synthase (CBS) and gamma-cystathionase (CTH)-use cysteine and/or homocysteine to produce the important signaling molecule hydrogen sulfide (H2S) and simultaneously the th