Mucopolysaccharidosis type IIIB (MPS IIIB), also known as Sanfilippo syndrome type B, is a metabolic disease caused by mutations in both alleles of the NAGLU gene encoding for the enzyme α-N-acetylglucosaminidase. A malfunction of this enzyme causes inability to degrade heparan sulfate, which leads to accumulation of glycosaminoglycans in the cells. MPS IIIB is associated with different symptoms such as neurodegeneration, extreme hyperactivity, sleeping problems, aggressive behavior, reduced fear, and cognitive deterioration. The condition is by now not curable. Here we describe a patient with MPS IIIB diagnosed at the age of 5 presenting with communication problems, motor dysfunctions, and speech and sleeping problems.Standard biochemical tests for neurodegenerative disorders and DNA analyses including NAGLU mutation screening were performed. We also did some psychological tests assessing the patient's communication skills and behavior. The patient was heterozygote for two mutations in the gene NAGLU (Y140C and Ser169fs). Thus, he suffered from MPS IIIB due to two mutations in the disease-causing gene.The patient presented with clear signs and symptoms of MPS IIIB with at least one of the two mutations affecting the α-N-acetylglucosaminidase protein function severely. Here we report the combination of a well-known and previously unreported mutation in the NAGLU gene; this could be dependent on geographical origin of the patient, which needs to be clarified by molecular studies of more MPS IIIB patients from Southeast Europe.
ABSTRACT Prior research has mainly focused on why individuals are engaging in sexting. However, little is known about sexting in intimate relationships, particularly sexting coercion. This study examined sexting coercion using social learning theory in a sample of young adults who had experience with a romantic relationship (N = 315, aged 14–28 years, 67.94% female). Individuals completed the sexting coercion scale and the social learning scale online. The results showed that over 33% of the respondents reported being victims of sexting coercion in an intimate relationship, while about 28% of them had perpetrated sexting coercion. The results suggest that sexting coercion in an intimate relationship is significantly predicted by differential association, differential reinforcement, and imitation. The findings of this study highlight the importance of exposing youth to evidence-based preventive educational interventions on sexting from the earliest age, based on the constructs of social learning theory. Practice Impact Statement The findings of this study of sexting coercion among young adults are relevant to educational programmes given recent evidence of the prevalence of sexting coercion victimisation and sexting coercion perpetration among youth in romantic relationships. The findings suggest that those who engage in sexting coercion use a social learning mechanism that should be considered when developing educational interventions to prevent sexting coercion.
Crna topola (Populus nigra L.) je jedna od najznačajnijih europskih vrsta drveća koja nastanjuje aluvijalna staništa uz obale velikih rijeka. Danas je jedna od najugroženijih vrsta šumskog drveća zbog devastacije staništa, regulacije riječnih tokova, i pretjerane ekspolatacije, kao i unošenja alohtonih vrsta drveća s kojima je hibridizirala. Cilj ovog istraživanja je utvrditi varijabilnost morfoloških svojstava listova crne topole unutar i između klonova autohtonih populacija i hibrida, te između riječnih slivova u Bosni i Hercegovini, kako bi se učinkovitije pristupilo zaštiti i oplemenjivanju ove vrste.Istraživan je materijal iz klonskog arhiva crnih topola. Arhiv je osnovan 2005. godine u Žepču, od klonova sa 161 stabla autohtonih crnih topola iz 26 populacija širom Bosne i Hercegovine (iz slivova 6 rijeka) te 15 hibridnih vrsta topola. Mjereno je po 5 listova od svakoga klona, a uziman je peti list od vrha glavnog izbojka. Mjerenje je vršeno digitalnim pomičnim mjerilom sa preciznošću od 0.1mm i kutomjerom. Mjereni su: dužina plojke, širina plojke, dužina peteljke, ukupna dužina lista, ugao insercije prvog bočnog nerva i centralnog nerva, udaljenost između najšireg dijela lista i baze lista, broj zubaca u dužini od 1cm iznad najšireg dijela lista. Podaci su obrađeni korištenjem SPSS 26.0 i EXCEL-a, i to prema populacijama i prema slivovima rijeka.Analizom varijance utvrđene su statistički značajne razlike između ispitivanih populacija, kao i između slivova rijeka, za sva istraživana svojstva. Najveću varijabilnost pokazalo je svojstvo dužine peteljke, a najmanju kut insercije prvog bočnog i centralnog nerva. Populacija dlakavih crnih topola Čapljina ima najmanje dimenzije listova i značajno odstupa u svim mjerenim svojstvima od ostalih. Najčešći broj zubaca na 1cm lisnog ruba je 4 zupca koji se pojavljuje na 38,3% listova. Ovo istraživanje daje mali uvid u morfološke karakteristike crnih topola u klonskom arhivu Žepče, te može predstavljati osnovu za daljnja istraživanja u cilju potpunijeg razumijevanja svojstava crnih topola potrebnih za uspješan nastavak rada na oplemenjivanju ove vrste.
Data-driven state estimation (SE) is becoming increasingly important in modern power systems, as it allows for more efficient analysis of system behaviour using real-time measurement data. This paper thoroughly evaluates a phasor measurement unit-only state estimator based on graph neural networks (GNNs) applied over factor graphs. To assess the sample efficiency of the GNN model, we perform multiple training experiments on various training set sizes. Additionally, to evaluate the scalability of the GNN model, we conduct experiments on power systems of various sizes. Our results show that the GNN-based state estimator exhibits high accuracy and efficient use of data. Additionally, it demonstrated scalability in terms of both memory usage and inference time, making it a promising solution for data-driven SE in modern power systems.
The microbiological aspects of traditional Travnik/Vlašić cheese was investigated. The cheese was made traditionally, from raw sheep milk at three small farms (A, B, C) on Mountain Vlašić. The microbiological quality of the cheese was examined during three stages of ripening (5, 30, 60 days) and followed during three seasons (3 years). Twenty-seven samples of cheese were collected and analyzed for the aerobic mesophilic count, yeasts and molds, coliforms and microorganisms from the group Staphylococcus spp. Average values determined for the number of investigated groups of microorganisms of all cheese samples throughout three different stages, seasons, and small farms were: aerobic mesophilic bacteria 8.03 log10 cfu·g-1, yeasts and molds 3.63 log10 cfu·g-1, coliforms 5.16 log10 cfu g-1, and microorganisms from the group Staphylococcus spp. 4.49 log10 cfu g-1. ANOVA showed that experimental factor ripening stage (days) had a significant effect on all testing parameters. Results obtained with this study indicate that hygiene during the production of traditional products must be increased in order to assure high quality of the final products.
Attending to the speech stream of interest in multi-talker environments can be a challenging task, particularly for listeners with hearing impairment. Research suggests that neural responses assessed with electroencephalography (EEG) are modulated by listener’s auditory attention, revealing selective neural tracking (NT) of the attended speech. NT methods mostly rely on hand-engineered acoustic and linguistic speech features to predict the neural response. Only recently, deep neural network (DNN) models without specific linguistic information have been used to extract speech features for NT, demonstrating that speech features in hierarchical DNN layers can predict neural responses throughout the auditory pathway. In this study, we go one step further to investigate the suitability of similar DNN models for speech to predict neural responses to competing speech observed in EEG. We recorded EEG data using a 64-channel acquisition system from 17 listeners with normal hearing instructed to attend to one of two competing talkers. Our data revealed that EEG responses are significantly better predicted by DNN-extracted speech features than by hand-engineered acoustic features. Furthermore, analysis of hierarchical DNN layers showed that early layers yielded the highest predictions. Moreover, we found a significant increase in auditory attention classification accuracies with the use of DNN-extracted speech features over the use of hand-engineered acoustic features. These findings open a new avenue for development of new NT measures to evaluate and further advance hearing technology.
Abstract This study aimed to research the effect of the seedless grape pomace (GP) added to feed mixture on meat quality, metabolic profile, and antioxidant status of Merinolandschaf lambs. The 90-day-old lambs in the control group (C, n=10) were fed feed mixture without GP while in experimental groups they were fed with 10% (GP10, n=10) or 20% (GP20, n=10) of GP in feed mixture for 30 days. There was a significant increase in L*, a* values and hue angle as well as a decrease in b* values of lamb carcasses in GP10 and GP20 compared to C group. When lambs were fed GP glucose concentrations decreased, while non-esterified fatty acids (NEFA) and β-hydroxybutyrate (BHB) increased on the 30th day in GP10 and GP20 compared with C group. A significant increase in SOD activity on day 30 and GPx activity on day 15 in lambs’ blood of GP10 and GP20 compared with C group was determined. Also, an increase in DPPH in lamb GP10 and GP20 compared with C was determined. The obtained results of the meat quality, metabolic profile and antioxidant status parameters of lamb meat showed that the use of 10% GP in the diet is justified, while 20% of GP was questionable.
Simple Summary The genus Sorbus (whitebeams, rowans, and service trees) encompasses forest trees and shrubs characterised by exceptional diversity resulting from the interplay of polyploidisation, hybridization, and apomixis. The spatiotemporal processes driving Sorbus diversification remain poorly understood. This research aims to provide insights into the evolution and diversification patterns of mountain whitebeam (S. austriaca) covering most of its range in the mountains of Central and South-eastern Europe. Our molecular and morphometric data revealed pronounced cryptic diversity within the S. austriaca complex; it is composed of different lineages, that likely originated via multiple allopolyploidisations accompanied by apomixes, and these lineages exhibit different distribution patterns. Our results are particularly valuable from a biodiversity conservation perspective due to the continuing generation of novel diversity in sympatric populations of the parental taxa. Such derived diversity requires process-oriented conservation plans and measures. Abstract The interplay of polyploidisation, hybridization, and apomixis contributed to the exceptional diversity of Sorbus (Rosaceae), giving rise to a mosaic of genetic and morphological entities. The Sorbus austriaca species complex from the mountains of Central and South-eastern Europe represents an allopolyploid apomictic system of populations that originated following hybridisation between S. aria and S. aucuparia. However, the mode and frequency of such allopolyploidisations and the relationships among different, morphologically more or less similar populations that have often been described as different taxa remain largely unexplored. We used amplified fragment length polymorphism (AFLP) fingerprinting, plastid DNA sequencing, and analyses of nuclear microsatellites, along with multivariate morphometrics and ploidy data, to disentangle the relationships among populations within this intricate complex. Our results revealed a mosaic of genetic lineages—many of which have not been taxonomically recognised—that originated via multiple allopolyploidisations. The clonal structure within and among populations was then maintained via apomixis. Our results thus support previous findings that hybridisation, polyploidization, and apomixis are the main drivers of Sorbus diversification in Europe.
Type 2 diabetes mellitus (T2DM) increases the risk of cardiovascular disease, especially myocardial injury. Due to their hypoglycemic effects, glucagon-like peptide-1 receptor agonists (GLP-1RAs) are efficiently used for T2DM management. GLP-1RAs also have anti-inflammatory and antioxidative effects, and can improve cardiac function. The aim of this study was to investigate the cardioprotective effects of liraglutide, a GLP-1RA, on isoprenaline-induced myocardial injury in rats. The study included 4 groups of animals. They were pretreated with saline for 10 days + saline on days 9 and 10 (control), saline for 10 days + isoprenaline on days 9 and 10 (isoprenaline group), liraglutide for 10 days + saline on days 9 and 10 (liraglutide group), and liraglutide for 10 days and on days 9 and 10 they were administered isoprenaline. This study evaluated ECG, myocardial injury markers, oxidative stress markers, and pathohistological changes. The results showed that liraglutide mitigated the isoprenaline-induced cardiac dysfunction recorded by ECG. Liraglutide reduced serum markers of myocardial injury such as high-sensitive troponin I, aspartate aminotransferase, alanine aminotransferase, reduced TBARS, increased catalase and superoxide dismutase activity and increased reduced glutathione, and improved lipid profile. Liraglutide induced antioxidative protection and alleviated isoprenaline-induced myocardial injury.
Observational studies play a critical role in evaluating the prevalence and incidence of conditions or diseases in populations as well as in defining the benefits and potential hazards of health-related interventions. There are currently no reporting guidelines for observational studies in the field of Endodontics. The Preferred Reporting Items for study Designs in Endodontology (PRIDE) team have developed and published new reporting guidelines for observational-based studies called the "Preferred Reporting items for OBservational studies in Endodontics (PROBE) 2023" guidelines. The PROBE 2023 guidelines were developed exclusively for the specialty of Endodontics by integrating and adapting the "STrengthening the Reporting of OBservational studies in Epidemiology (STROBE)" checklist and the "Clinical and Laboratory Images in Publications (CLIP)" principles. The recommendations of the Guidance for Developers of Health Research Reporting Guidelines were adhered to throughout the process of developing the guidelines. The purpose of this document is to serve as a guide for authors by providing an explanation for each of the items in the PROBE 2023 checklist along with relevant examples from the literature. The document also offers advice to authors on how they can address each item in their manuscript before submission to a journal. The PROBE 2023 checklist is freely accessible and downloadable from the Preferred Reporting Items for study Designs in Endodontology (PRIDE) website (http://pride-endodonticguidelines.org/probe/).
This article explores a possibility to improve mathematical teaching by using 3D printing technology. The question is whether it is possible to use low cost additive manufacturing technology to develop and manufacture real physical prototypes of complex mathematical surfaces and volumes and on that way improve mathematics education. Five mathematical problems were chosen as case studies. Visualization of this problems was done using professor hand drawing, using computer visualization and using development and manufacturing of real physical prototypes. To find out how much better is understanding of these problems, survey with 57 students is carried out. Results showed significant improvements of understanding and better visualization of selected mathematical problems.
Abstract This study aimed to investigate the effects of dietary supplementation with modified-hen-egg-yolk on plasma lipids and lipoprotein profiles in rats. During the four-week-experiment, 64 Wistar rats were divided into four groups of 16 (eight of both sexes), and fed commercial rat food (group C); food containing 70% commercial rat mixture and 30% freshly cooked egg yolk originating from laying hen eggs fed with 3% fish oil (group F); 3% palm olein (group P), or 3% lard (group L). The cooked egg yolk in the rat diet affected the concentrations of plasma total and LDL-cholesterol in males of the P and L groups. Cholesterol and total fat in the diet did not have a hypercholesterolemic effect on their own, but when in combination with fatty acid composition, they could contribute to an increase in plasma total and LDL cholesterol concentrations in rats. HDL-cholesterol was the most resilient plasma lipoprotein of rats to dietary treatments in our experiment. Compared to the control group, the addition of hen egg yolk to the rat diet regardless of its quality, adversely affected the values of HDL-C/TC and HDL-C/LDL-C in both males and females.
Mycoplasma ovipneumoniae is an important pathogen in sheep, goats, and wild ruminants. We sequenced M. ovipneumoniae strains 150 and 274 from Bosnia and Herzegovina. Strain 150 has a circular genome of 1,053,380 bp with 29.15% GC content while strain 274 has 1,081,404 bp with 28.82% GC content. ABSTRACT Mycoplasma ovipneumoniae is an important pathogen in sheep, goats, and wild ruminants. We sequenced M. ovipneumoniae strains 150 and 274 from Bosnia and Herzegovina. Strain 150 has a circular genome of 1,053,380 bp with 29.15% GC content while strain 274 has 1,081,404 bp with 28.82% GC content.
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