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Zeman J

From Bioblast
Name Zeman Jiří, Prof.MUDr DrSc PhD
Institution Department of Pediatrics

First Faculty of Medicine

Charles University

Address Ke Karlovu 2, 121 09
City Prague
State/Province
Country Czech Republic
Email [email protected]
Weblink
O2k-Network Lab CZ Prague Zeman J


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Publications

 PublishedReference
Burska 2021 Biochim Biophys Acta Mol Basis Dis2021Burska D, Stiburek L, Krizova J, Vanisova M, Martinek V, Sladkova J, Zamecnik J, Honzik T, Zeman J, Hansikova H, Tesarova M (2021) Homozygous missense mutation in UQCRC2 associated with severe encephalomyopathy, mitochondrial complex III assembly defect and activation of mitochondrial protein quality control. Biochim Biophys Acta Mol Basis Dis 1867:166147.
Rodinova 2019 Dis Model Mech2019Rodinova M, Krizova J, Stufkova H, Bohuslavova B, Askeland G, Dosoudilova Z, Juhas S, Juhasova J, Ellederova Z, Zeman J, Eide L, Motlik J, Hansikova H (2019) Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease. Dis Model Mech 12:dmm038737.
Fisar 2016 Curr Alzheimer Res2016Fišar Z, Hroudová J, Hansíková H, Spáčilová J, Lelková P, Wenchich L, Jirák R, Zvěřová M, Zeman J, Martásek P, Raboch J (2016) Mitochondrial respiration in the platelets of patients with Alzheimer's disease. Curr Alzheimer Res 13:930-41.
Stiburek 2012 Mol Biol Cell2012Stiburek L, Cesnekova J, Kostkova O, Fornuskova D, Vinsova K, Wenchich L, Houstek J, Zeman J (2012) YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis and cell proliferation. Mol Biol Cell 23: 1010-1023.
Fornunskova 2010 Biochem J2010Fornuskova D, Stiburek L, Wenchich L, Vinsova K, Hansikova H, Zeman J (2010) Novel insights into the assembly and function of human nuclear-encoded cytochrome c oxidase subunits 4, 5a, 6a, 7a and 7b. Biochem J 428:363-74.
Cizkova 2008 Nat Genet2008Cızkova A, Stranecky V, Mayr JA, Tesarova M, Havlıckova V, Paul J, Ivanek R, Kuss AW, Hansıkova H, Kaplanova W, Vrbacky M, Hartmannova H, Noskova L, Honzık T, Drahota Z, Magner M, Hejzlarova K, Sperl W, Zeman J, Houstek J, Kmoch S (2008) TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy. Nat Genet 40:1288-90.
Honzik 2006 Placenta2006Honzik T, Drahota Z, Bohm M, Jesina P, Mracek T, Paul J, Zeman J, Houstek J (2006) Specific properties of heavy fraction of mitochondria from human-term placenta - Glycerophosphate-dependent hydrogen peroxide production. Placenta 27:348-56.
Jesina 2004 Biochem J2004Jesina P, Tesarova M, Fornuskova D, Vojtiskova A, Pecina P, Kaplanova V, Hansikova H, Zeman J, Houstek J (2004) Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206. Biochem J 383:561-71.
Pecina 2004 Physiol Res2004Pecina P, Houstkova H, Hansikova H, Zeman J, Houstek J (2004) Genetic defects of cytochrome c oxidase assembly. Physiol Res 53 Suppl 1:S213-23.
Pecina 2004 Am J Physiol Cell Physiol2004Pecina P, Gnaiger E, Zeman J, Pronicka E, Houstek J (2004) Decreased affinity to oxygen of cytochrome c oxidase in Leigh syndrome caused by SURF1 mutations. Am J Physiol Cell Physiol 287:C1384-8.
Pecina 2003 Biochim Biophys Acta2003Pecina P, Capkova M, Chowdhury SK, Drahota Z, Dubot A, Vojtiskova A, Hansikova H, Houstekova H, Zeman J, Godinot C, Houstek J (2003) Functional alteration of cytochrome c oxidase by SURF1 mutations in Leigh syndrome. Biochim Biophys Acta 1639:53-63.
Wenchich 2003 Physiol Res2003Wenchich L, Drahota Z, Honzík T, Hansíková H, Tesarová M, Zeman J, Houstek J (2003) Polarographic evaluation of mitochondrial enzymes activity in isolated mitochondria and in permeabilized human muscle cells with inherited mitochondrial defects. Physiol Res 52:781-88.

Abstracts

 PublishedReference
Danhelovska 2018 MiP20182018
MiPsociety
The relevance of ACBD3 protein in energy metabolism in various cell lines. Danhelovska_Presentation
Zdrazilova 2018 MiPschool Tromso C42018
MiPsociety
Bioenergetic characterization of skin fibroblasts from patients with congenital disorders of glycosylation.
Krizova 2016 Abstract IOC1162016Krizova J, Macakova M, Bohuslavova B, Klima J, Ellederova Z, Zeman J, Motlik J, Hansikova H (2016) Complex I-dependent respiration decline is a marker of Huntington`s disease in spermatozoa of transgenic minipig model. Mitochondr Physiol Network 21.11
Rodinova 2015 Abstract MiP20152015Altered mitochondrial ultrastructure, reduced respiration and decreased level of PDH subunits in fibroblasts from 10 patients with Huntington’s disease.
Kratochvilova 2015 Abstract MiP20152015Mitochondrial dysfunction in Niemann Pick type C1 patient´s cells and tissues.
Pecina 2014 Abstract MiP20142014Manifestation of mitochondrial disorders of nuclear origin in lymphocytes.
Pecina 2013 Abstract MiP20132013Pecina P, Houšťková H, Mráček T, Pecinová A, Nůsková H, Tesařová M, Hansíková H, Janota J, Zeman J, Houštěk J (2013) The use of lymphocytes for diagnostics of mitochondrial oxidative phosphorylation disorders. Mitochondr Physiol Network 18.08.