Research Topics
Species | Jiri ZemanSummaryAffiliation: Charles University Country: Czech Republic Publications
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Detail Information
Publications
Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare diseaseMaurizio Scarpa
Department of Pediatrics, University of Padua, Padua, Italy
Orphanet J Rare Dis 6:72. 2011..Take-home message: Expertise in recognising and treating patients with MPS II varies widely between countries. This article presents pan-European recommendations for the diagnosis and management of this life-limiting disease...
Development of a human mitochondrial oligonucleotide microarray (h-MitoArray) and gene expression analysis of fibroblast cell lines from 13 patients with isolated F1Fo ATP synthase deficiencyAlena Cízková
Center for Applied Genomics, 1st Faculty of Medicine, Charles University, Prague, Czech Republic
BMC Genomics 9:38. 2008....
[History of foster care in the Czech Republic]J Zeman
Klinika detského a dorostového lékarství 1 LF UK a VFN, Praha
Cas Lek Cesk 145:673-4. 2006..The law incorporated ten principles how to provide, control and finance the foster care in our region...
Assembly factors and ATP-dependent proteases in cytochrome c oxidase biogenesisLukas Stiburek
Charles University in Prague, First Faculty of Medicine, Department of Pediatrics, Prague, Czech Republic
Biochim Biophys Acta 1797:1149-58. 2010..In this review, we summarize the current knowledge of the functional involvement of eukaryotic CcO-specific assembly factors and highlight the possible significance for CcO biogenesis of mitochondrial ATP-dependent proteases...
Cytosine-Adenosine (CA)n repeats polymorphism in IGF-I gene and early growth in infants born appropriate and small for gestational ageJitka Kytnarova
Department of Paediatrics, Charles University in Prague, Czech Republic
Neuro Endocrinol Lett 30:501-5. 2009..IGF-I gene polymorphisms might alter IGF-I level resulting in decreased foetal and postnatal growth and increased risk for diabetes mellitus type 2 and cardiovascular diseases in adulthood...
Biochemical and molecular analyses in three patients with 3-hydroxy-3-methylglutaric aciduriaE Pospisilova
Institute of Inherited Metabolic Diseases, General Faculty Hospital and 1st Faculty of Medicine, Prague, Czech Republic
J Inherit Metab Dis 26:433-41. 2003..This study expands the knowledge of the genotypic variability of the HMG aciduria...
Eight novel ABCD1 gene mutations and three polymorphisms in patients with X-linked adrenoleukodystrophy: The first polymorphism causing an amino acid exchangeL Dvorakova
Institute for Inherited Metabolic Disorders, First Faculty of Medicine and General Faculty Hospital, Prague, Czech Republic
Hum Mutat 18:52-60. 2001..59 C/T in the 5'untranslated region and c.2019 C/T (F673F) in exon 10. The frequencies of these two polymorphisms, were 11/150 and 2/150 control alleles, respectively...
Maternal hyperphenylalaninemias in healthy Czech population of pregnant women: 30 years experience with screening, prevention and treatmentJ Hyanek
Institute for Inherited Metabolic Disorders, Obstetric and Gynaecologic Clinic of 1st Medical School Charles University Prague, Czech Republic
Bratisl Lek Listy 105:291-8. 2004..The toxic effect causes spontaneous abortion or retards intrauterine growth, skeletal malformation, cardiac anomalies can appear. However the most frequent are microcephaly, mental retardation and hypotrophy...
Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach studyH Pavlu-Pereira
Institute of Inherited Metabolic Disorders, Prague, Czech Republic
J Inherit Metab Dis 28:203-27. 2005..It appears more common in central Europe. The visceral, rapidly progressing early fatal type has been recognized rarely so far...
