Lignocellulosic Bioethanol and Biobutanol as a Biocomponent for Diesel Fuel.

In this paper, the fuel properties of mixtures of diesel fuel and ethanol and diesel fuel and butanol in the ratio of 2.5% to 30% were investigated. The physicochemical properties of the blends such as the cetane number, cetane index, density, flash point, kinematic viscosity, lubricity, CFPP, and distillation characteristics were measured, and the effect on fuel properties was evaluated.

Use of Dicyclopentadiene and Methyl Dicyclopentadiene for the Synthesis of Unsaturated Polyester Resins.

Dicyclopentadiene (DCPD) modified unsaturated polyester resins (UPRs) are mostly used for hulls, decks, and bathroom facilities. Main advantages of these polymers over orthophthalic or isophthalic polyesters are their relatively low shrinking, reduced styrene emission, lower cost, and fast curing in thin layers. On the other hand, once cured, these materials are more brittle and have lower glass transition temperatures and lower chemical resistance due to their different chemical constitutions.

Advanced Biofuels Based on Fischer-Tropsch Synthesis for Applications in Gasoline Engines.

The aim of the article is to determine the properties of fuel mixtures of Fischer-Tropsch naphtha fraction with traditional gasoline (petrol) to be able to integrate the production of advanced alternative fuel based on Fischer-Tropsch synthesis into existing fuel markets. The density, octane number, vapor pressure, cloud point, water content, sulphur content, refractive index, ASTM color, heat of combustion, and fuel composition were measured using the gas chromatography method PIONA. It was found that fuel properties of Fischer-Tropsch naphtha fraction is not much comparable to conventional gasoline (petrol) due to the high n-alkane content.

Advanced Biofuels Based on Fischer-Tropsch Synthesis for Applications in Diesel Engines.

This paper focuses on the evaluation of the fuel properties of Fischer-Tropsch diesel blends with conventional diesel. Incorporating this advanced fuel into conventional diesel production will enable the use of waste materials and non-food materials as resources, while contributing to a reduction in dependence on crude oil. To evaluate the suitability of using Fischer-Tropsch diesel, cetane number, cetane index, CFPP, density, flash point, heat of combustion, lubricity, viscosity, distillation curve, and fuel composition ratios using multidimensional GC × GC-TOFMS for different blends were measured.

Spectrum and frequencies of non GJB2 gene mutations in Czech patients with early non-syndromic hearing loss detected by gene panel NGS and whole-exome sequencing.

Non-syndromic autosomal recessive hearing loss is an extremely heterogeneous disease caused by mutations in more than 80 genes. We examined Czech patients with early/prelingual non-syndromic, presumably genetic hearing loss (NSHL) without known cause after GJB2 gene testing. Four hundred and twenty-one unrelated patients were examined for STRC gene deletions with quantitative comparative fluorescent PCR (QCF PCR), 197 unrelated patients with next-generation sequencing by custom-designed NSHL gene panels and 19 patients with whole-exome sequencing (WES).

Variant c.2158-2A>G in MANBA is an important and frequent cause of hereditary hearing loss and beta-mannosidosis among the Czech and Slovak Roma population- evidence for a new ethnic-specific variant.

Background: The Roma are a European ethnic minority threatened by several recessive diseases. Variants in MANBA cause a rare lysosomal storage disorder named beta-mannosidosis whose clinical manifestation includes deafness and mental retardation. Since 1986, only 23 patients with beta-mannosidosis and biallelic MANBA variants have been described worldwide.

Two types of recessive hereditary spastic paraplegia in Roma patients in compound heterozygous state; no ethnically prevalent variant found.

Hereditary spastic paraplegia (HSP or SPG) is a group of rare upper motor neuron diseases. As some ethnically-specific, disease-causing homozygous variants were described in the Czech Roma population, we hypotesised that some prevalent HSP-causing variant could exist in this population. Eight Czech Roma patients were found in a large group of Czech patients with suspected HSP and were tested using gene panel massively parallel sequencing (MPS).

Moderate sensorineural hearing loss is typical for DFNB16 caused by various types of mutations affecting the STRC gene.

Introduction: Hearing loss is the most frequent sensory disorder and is genetically extremely heterogeneous. By far the most frequent cause of nonsyndromic autosomal recessive hearing loss (AR-NSHL) are biallelic pathogenic mutations in the GJB2 gene causing DFNB1. The worldwide search for the second most common type of AR-NSHL took almost two decades.

Fanconi anemia with biallelic FANCD1/BRCA2 mutations - Case report of a family with three affected children.

Fanconi anemia, complementation group D1 with bi-allelic FANCD1 (BRCA2) mutations, is a very rare genetic disorder characterized by early onset of childhood malignancies, including acute leukemia, brain cancer and nephroblastoma. Here, we present a case report of a family with 3 affected children in terms of treatment outcome, toxicity and characterization of the malignancies using comprehensive cytogenetic analysis. The first child was diagnosed with T-cell acute lymphoblastic leukemia when he was 11 months old.