Decomposition analysis of global value chains: Uncovering vulnerable spots in a selected economy (the example of Czechia).

Recent global turmoil has added to the importance of food security issues and the sustainability of production processes worldwide. This study aims to uncover the degree of domestic industries' dependence on the international fragmentation of production processes and identify the countries of origin of those producers who have managed to displace domestic producers in corresponding Global Value Chains. Using data retrieved from the World Input-Output Database, we studied Czechia's example via demarcation of domestic value-added (DVA) shares from foreign ones in final domestic products.

Energy Logistic Regression and Survival Model: Case Study of Russian Exports.

The importance of environmental sustainability is becoming more and more obvious, so the rationale behind long-term usage of solely non-renewable energy sources appeared questionable. This study aims to identify, using Kaplan-Meier survival analysis and logistic regressions, the main determinants that affect the duration of Russian non-renewable energy exports to different regions of the world. Data were retrieved from the databanks of the World Development Indicators (WDI), World Integrated Trade Solution (WITS), and the French Centre for Prospective studies and International Information (CEPII).

Investigating the dynamic eco-efficiency in agriculture sector of the European Union countries.

One of the main aims of European Common Agricultural Policy (CAP) has been established on a considerable reduction of greenhouse gas (GHG) emissions without any decline in productivity by 2030. This introduces some challenges in the assessment of agricultural technical efficiency. In particular, we can formulate at least two problems that are worth to be addressed: (i) possibility of treating undesirable outputs and (ii) panel data analysis of agricultural technical efficiency enriched by eco-efficiency assessment.

Lentiviral gene therapy vector with UCOE stably restores function in iPSC-derived neutrophils of a CDG patient.

A recent gamma-retroviral clinical Chronic Granulomatous Disease (CGD) gene therapy (GT) trial achieved proof-of-concept but was accompanied by activation of oncogenes and transgene silencing. The (UCOE) comprises the sequences of two divergently oriented house-keeping gene promoters and is known to have anti-silencing properties. In a screen we identified two novel UCOE constructs that prevent adjacent promoter methylation in P cells.

Severe glucose-6-phosphate dehydrogenase deficiency leads to susceptibility to infection and absent NETosis.

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymatic disorder of red blood cells in human subjects, causing hemolytic anemia linked to impaired nicotinamide adenine dinucleotide phosphate (NADPH) production and imbalanced redox homeostasis in erythrocytes. Because G6PD is expressed by a variety of hematologic and nonhematologic cells, a broader clinical phenotype could be postulated in G6PD-deficient patients. We describe 3 brothers with severe G6PD deficiency and susceptibility to bacterial infection.

Successful Combination of Sequential Gene Therapy and Rescue Allo-HSCT in Two Children with X-CGD - Importance of Timing.

We report on a series of sequential events leading to long-term survival and cure of pediatric X-linked chronic granulomatous disease (X-CGD) patients after gamma-retroviral gene therapy (GT) and rescue HSCT. Due to therapyrefractory life-threatening infections requiring hematopoietic stem cell transplantation (HSCT) but absence of HLAidentical donors, we treated 2 boys with X-CGD by GT. Following GT both children completely resolved invasive Aspergillus nidulans infections.

Human miR223 promoter as a novel myelo-specific promoter for chronic granulomatous disease gene therapy.

Targeting transgene expression to specific hematopoietic cell lineages could contribute to the safety of retroviral vectors in gene therapeutic applications. Chronic granulomatous disease (CGD), a defect of phagocytic cells, can be managed by gene therapy, using retroviral vectors with targeted expression to myeloid cells. In this context, we analyzed the myelospecificity of the human miR223 promoter, which is known to be strongly upregulated during myeloid differentiation, to drive myeloid-restricted expression of p47(phox) and gp91(phox) in mouse models of CGD and in primary patient-derived cells.

Association of hypoalbuminemia with the presence and size of pleural effusion in children with pneumonia.

Objective: Hypoalbuminemia is a common finding in children with massive parapneumonic pleural effusion; however, its incidence and pathogenesis are unclear. The objective of this study was to assess the presence and severity of hypoalbuminemia in children with parapneumonic pleural effusion and to propose a possible pathophysiologic mechanism.

Methods: The clinical charts of patients who were hospitalized in a tertiary pediatric center with bacterial pneumonia complicated by pleural effusion were reviewed.

Caveolae: biochemical analysis.

Caveolae appear in a multitude of processes encompassing growth regulation and trafficking. We demonstrate the abundant presence of ESA/reggie-1/flotillin-2, ATP synthase beta subunit and annexin V in endothelial caveolae by immunopurification of caveolae from vascular endothelial membrane. Five proteins are abundant in a caveolin-1 protein complex, analyzed by sucrose gradient velocity sedimentation following octyl-beta-D-glucopyranoside extraction.

LDL transcytosis by protein membrane diffusion.

Endothelial cell (EC) cultures of different, selected vascular beds and/or organs were screened for receptor-mediated transport of proteins with a semipermeable filter assay. In SVEC4-10 cells, a mouse lymphoid endothelial cell line, orosomucoid, albumin, insulin and LDL were transcytosed from the apical (luminal) to basal (abluminal) side by a receptor-mediated pathway. Specific LDL transcytosis involved transport of intact LDL.