Determination of S-Adenosylmethionine and S-Adenosylhomocysteine by LC–MS/MS and evaluation of their stability in mice tissues

gan Krijt, Jakub, Dutá, Alena, Kožich, Viktor
Cyhoeddwyd 2009

Restoring assembly and activity of cystathionine β-synthase mutants by ligands and chemical chaperones

gan Kopecká, Jana, Krijt, Jakub, Raková, Kateřina, Kožich, Viktor
Cyhoeddwyd 2010

Novel structural arrangement of nematode cystathionine β-synthases: characterization of Caenorhabditis elegans CBS-1

gan Vozdek, Roman, Hnízda, Aleš, Krijt, Jakub, Kostrouchová, Marta, Kožich, Viktor
Cyhoeddwyd 2012

Birth Prevalence of Homocystinuria in Central Europe: Frequency and Pathogenicity of Mutation c.1105C>T (p.R369C) in the Cystathionine Beta-Synthase Gene

gan Janošík, Miroslav, Sokolová, Jitka, Janošíková, Bohumila, Krijt, Jakub, Klatovská, Veronika, Kožich, Viktor
Cyhoeddwyd 2009

Cystathionine β-Synthase p.S466L Mutation Causes Hyperhomocysteinemia in Mice

gan Gupta, Sapna, Wang, Liqun, Hua, Xiang, Krijt, Jakub, Kožich, Viktor, Kruger, Warren D.
Cyhoeddwyd 2008

Metabolism of sulfur compounds in homocystinurias

gan Kožich, Viktor, Ditrói, Tamás, Sokolová, Jitka, Křížková, Michaela, Krijt, Jakub, Ješina, Pavel, Nagy, Peter
Cyhoeddwyd yn Br J Pharmacol (2018)

The Cysteine Dioxgenase Knockout Mouse: Altered Cysteine Metabolism in Nonhepatic Tissues Leads to Excess H(2)S/HS(−) Production and Evidence of Pancreatic and Lung Toxicity

gan Roman, Heather B., Hirschberger, Lawrence L., Krijt, Jakub, Valli, Alessandro, Kožich, Viktor, Stipanuk, Martha H.
Cyhoeddwyd 2013

Enzyme replacement prevents neonatal death, liver damage, and osteoporosis in murine homocystinuria

gan Majtan, Tomas, Hůlková, Helena, Park, Insun, Krijt, Jakub, Kožich, Viktor, Bublil, Erez M., Kraus, Jan P.
Cyhoeddwyd yn FASEB J (2017)

Cystathionine β-Synthase Mutations: Effect of Mutation Topology on Folding and Activity

gan Kožich, Viktor, Sokolová, Jitka, Klatovská, Veronika, Krijt, Jakub, Janošík, Miroslav, Jelínek, Karel, Kraus, Jan P, Cooper, David N
Cyhoeddwyd 2010

Enzyme replacement with PEGylated cystathionine β-synthase ameliorates homocystinuria in murine model

gan Bublil, Erez M., Majtan, Tomas, Park, Insun, Carrillo, Richard S., Hůlková, Helena, Krijt, Jakub, Kožich, Viktor, Kraus, Jan P.
Cyhoeddwyd yn J Clin Invest (2016)

Primary hepatocytes from mice lacking cysteine dioxygenase show increased cysteine concentrations and higher rates of metabolism of cysteine to hydrogen sulfide and thiosulfate

gan Jurkowska, Halina, Roman, Heather B., Hirschberger, Lawrence L., Sasakura, Kiyoshi, Nagano, Tetsuo, Hanaoka, Kenjiro, Krijt, Jakub, Stipanuk, Martha H.
Cyhoeddwyd yn Amino Acids (2014)

Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria

gan Majtan, Tomas, Jones, Wendell, Krijt, Jakub, Park, Insun, Kruger, Warren D., Kožich, Viktor, Bassnett, Steven, Bublil, Erez M., Kraus, Jan P.
Cyhoeddwyd yn Mol Ther (2018)

Folate Deficiency Is Associated With Oxidative Stress, Increased Blood Pressure, and Insulin Resistance in Spontaneously Hypertensive Rats

gan Pravenec, Michal, Kožich, Viktor, Krijt, Jakub, Sokolová, Jitka, Zídek, Václav, Landa, Vladimír, Šimáková, Miroslava, Mlejnek, Petr, Šilhavý, Jan, Oliyarnyk, Olena, Kazdová, Ludmila, Kurtz, Theodore W.
Cyhoeddwyd 2013

Cystathionine β‐synthase deficiency in the E‐HOD registry‐part I: pyridoxine responsiveness as a determinant of biochemical and clinical phenotype at diagnosis

gan Kožich, Viktor, Sokolová, Jitka, Morris, Andrew A. M., Pavlíková, Markéta, Gleich, Florian, Kölker, Stefan, Krijt, Jakub, Dionisi‐Vici, Carlo, Baumgartner, Matthias R., Blom, Henk J., Huemer, Martina
Cyhoeddwyd yn J Inherit Metab Dis (2020)

Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment

gan Maclean, Kenneth N., Sikora, Jakub, Kožich, Viktor, Jiang, Hua, Greiner, Lori S., Kraus, Eva, Krijt, Jakub, Crnic, Linda S., Allen, Robert H., Stabler, Sally P., Elleder, Milan, Kraus, Jan P.
Cyhoeddwyd 2010

A homozygous deletion in the SLC19A1 gene as a cause of folate-dependent recurrent megaloblastic anemia

gan Svaton, Michael, Skvarova Kramarzova, Karolina, Kanderova, Veronika, Mancikova, Andrea, Smisek, Petr, Jesina, Pavel, Krijt, Jakub, Stiburkova, Blanka, Dobrovolny, Robert, Sokolova, Jitka, Bakardjieva-Mihaylova, Violeta, Vodickova, Elena, Rackova, Marketa, Stuchly, Jan, Kalina, Tomas, Stary, Jan, Trka, Jan, Fronkova, Eva, Kozich, Viktor
Cyhoeddwyd yn Blood (2020)

A novel transgenic mouse model of CBS-deficient homocystinuria does not incur hepatic steatosis or fibrosis and exhibits a hypercoagulative phenotype that is ameliorated by betaine...

gan Maclean, Kenneth N., Sikora, Jakub, Kožich, Viktor, Jiang, Hua, Greiner, Lori S., Kraus, Eva, Krijt, Jakub, Overdier, Katherine H., Collard, Renata, Brodsky, Gary L., Meltesen, Lynne, Crnic, Linda S., Allen, Robert H., Stabler, Sally P., Elleder, Milan, Rozen, Rima, Patterson, David, Kraus, Jan P.
Cyhoeddwyd 2010