Bücher veröffentlicht von Mdpi AG

Significant scientific and therapeutic advances have been made in recent decades, particularly in hereditary but also in acquired neuromuscular diseases. As a result of our increasing etiological understanding, the classification of these diseases has changed from a clinical-descriptive and formal-genetic to a molecular-genetic and pathophysiological one. This has led to an intensification of research into the diagnosis and treatment of these diseases, resulting in the first effective gene-modifying treatments for DMD and SMA in recent years and, more recently, gene replacement therapy for the most severe form of SMA. In addition, great strides have been made in symptomatic and rehabilitative treatment, making it possible to improve the functioning and quality of life of those affected and their families. This Special Issue of Children contains a collection of 12 studies and reviews dealing with genetic and acquired peripheral nerve and muscle disorders.

This reprint presents some recent results from applying original analytical methods to the most renowned hive matrices. Particular consideration was given to methods devoted to the attribution of the origin of honey and propolis, but also studies dealing with the chemical characterization of honey and other hive matrices are here reported. Attention has also been paid to the use of optimized methods of elemental analysis in several hive products for quality and safety purposes, but also for environmental biomonitoring. The treatment of the data was often achieved through multivariate analysis methods, which made it possible to obtain reliable classifications of honeys and propolis according to their botanic or geographical origin.

Since its early introduction by the Russian botanist Mikhail Semyonovich Tsvet, chromatography has been undoubtedly the most powerful analytical tool in analytical chemistry. Separation, qualitative analysis, and quantitative analysis can be achieved by choosing the right conditions. Thus, numerous gas chromatographic, liquid chromatographic, and supercritical fluid chromatographic methods have been developed and applied for most types of samples and most kinds of analytes. Additionally, older varieties such as paper chromatography and thin-layer chromatography were pioneer analytical techniques in many laboratories. Especially when hyphenated to spectrometric techniques, chromatography also allows the identification of separated analytes in a single run. Highly sophisticated equipment can answer all analytical problems very quickly. Chromatographers cooperate with many scientific fields and give their lights to medical doctors, veterinarians, food scientists, biologists, dentists, archaeologists, etc. In this Special Issue, analytical chemists were invited to prove that chromatography-based separation techniques are the ultimate analytical tool and their significant contribution is reflected in ten interesting articles.

The purpose of this reprint is to discuss the current state, problems and prospects of hydrocarbon resources development on the Arctic shelf. This book examines economic, ecological, geological, and technological aspects of Arctic marine oil and gas resources exploration and development. Important issues covered in the book are safety and ensuring the sustainability of the energy sector. Studies presented in the book address issues of ecologically balanced and socially sustainable development of marine and coastal territories of the Arctic.

In recent years, the formulation of innovative photocatalysts activated by visible or solar light has been attracting increasing attention because of their notable potential for environmental remediation and use in organic synthesis reactions. Generally, the strategies for the development of visible-light-active photocatalysts are mainly focused on enhancing degradation efficiency (in the case of environmental remediation) or increasing selectivity toward the desired product (in the case of organic synthesis). These goals can be achieved by doping the semiconductor lattice with metal and/or non-metal elements in order to reduce band gap energy, thereby providing the semiconductor with the ability to absorb light at a wavelength higher than the UV range. Other interesting options are the formulation of different types of heterojunctions (to increase visible absorption properties and to reduce the recombination rate of charge carriers) and the development of innovative catalytic materials with semiconducting properties. This reprint is focused on visible-light-active photocatalysts for environmental remediation and organic synthesis, featuring the state of the art as well as advances in this field.

Antimicrobial-resistant bacterial infections are a major and costly public health concern. Several pathogens are already pan-resistant, representing a major cause of mortality in patients suffering from nosocomial infections. Drug efflux pumps, which remove compounds from the bacterial cell, thereby lowering the antimicrobial concentration to sub-toxic levels, play a major role in multidrug resistance. In this Special Issue, we present up-to-date knowledge of the mechanism of RND efflux pumps, the identification and characterization of efflux pumps from emerging pathogens and their role in antimicrobial resistance, and progress made on the development of specific inhibitors. This collection of data could serve as a basis for antimicrobial drug discovery aimed at inhibiting drug efflux pumps to reverse resistance in some of the most resistant pathogens.

The Identification of the Genetic Components of Autism Spectrum Disorders 2020 will be a useful resource for laboratory and clinical scientists, translational-based researchers, primary healthcare providers and physicians, psychologists/psychiatrists, neurologists, developmental pediatricians, clinical geneticists, teachers, special educators, and caregivers involved with individuals who have autism spectrum disorders (ASD), with the goal to translate information directly to the clinical, education and home settings. Other professionals, students at all levels, and families who are interested in this important neurodevelopmental disorder will find this textbook of value by obtaining a better awareness of the causes, testing, and understanding of genetic components leading to autism, and research that may open avenues for treatment with new approaches. This textbook includes nine chapters divided into three sections (clinical, genetics, other) written by experts in the field dedicated to genetics research and clinical care, description, and treatment by generating reviews for ASD and related disorders. These chapters include information on discoveries, risk factors, causation, diagnosis, treatment, and phenotyping with characterization of genomic or genetic factors and the environment, as genetics play an important role in up to 90% of individuals with autism via over 800 currently recognized genes.

Three of the eleven papers focused on groundwater recharge and its impacts on the groundwater regime, in which recharge was caused by riverbed leakage from river ecological restoration (artificial water replenishment). The issues of the hydrogeological parameters involved (such as the influence radius) were also reconsidered. Six papers focused on the impact of river ecological replenishment and other human activities on river and watershed ecology, and on groundwater quality and use function. The issues of ecological security at the watershed scale and deterioration of groundwater quality were of particular concern. Two papers focused on water resources carrying capacity and water resources reallocation at the regional scale, in the context of the fact that ecological water demand has been a significant topic of concern. The use of unconventional water resources such as brackish water has been emphasized in the research in this issue.

Mammalian ATP-binding cassette (ABC) transporters constitute a superfamily of proteins involved in many essential cellular processes. Most of these transporters are transmembrane proteins and allow the active transport of solutes, small molecules, and lipids across biological membranes. On the one hand, some of these transporters are involved in drug resistance (also referred to as MDR or multidrug resistance), a process known to be a major brake in most anticancer treatments, and the medical challenge is thus to specifically inhibit their function. On the other hand, molecular defects in some of these ABC transporters are correlated with several rare human diseases, the most well-documented of which being cystic fibrosis, which is caused by genetic variations in ABCC7/CFTR (cystic fibrosis transmembrane conductance regulator). In the latter case, the goal is to rescue the function of the deficient transporters using various means, such as targeted pharmacotherapies and cell or gene therapy. The aim of this Special Issue, "ABC Transporters in Human Diseases", is to present, through original articles and reviews, the state-of-the-art of our current knowledge about the role of ABC transporters in human diseases and the proposed therapeutic options based on studies ranging from cell and animal models to patients.

The worldwide dissemination of antimicrobial-resistant bacteria, particularly those resistant to last-resource antibiotics, is a common problem to which no immediate solution is foreseen. In 2017, the World Health Organization (WHO) published a list of antimicrobial-resistant "priority pathogens", which include a group of microorganisms with high-level resistance to multiple drugs, named ESKAPE pathogens, comprising vancomycin-resistant Enterococcus faecium (VRE), methicillin- and vancomycin-resistant Staphylococcus aureus (MRSA and VRSA), extended spectrum ¿-lactamase (ESBL) or carbapenem-resistant Klebsiella pneumoniae, carbapenem-resistant Acinetobacter baumannii, carbapenem-resistant Pseudomonas aeruginosa and extended spectrum ¿-lactamase (ESBL) or carbapenem-resistant Enterobacter spp. These bacteria also have the ability to produce several virulence factors, which have a major influence on the outcomes of infectious diseases. Bacterial resistance and virulence are interrelated, since antibiotics pressure may influence bacterial virulence gene expression and, consequently, infection pathogenesis. Additionally, some virulence factors contribute to an increased resistance ability, as observed in biofilm-producing strains. The surveillance of important resistant and virulent clones and associated mobile genetic elements is essential to decision making in terms of mitigation measures to be applied for the prevention of such infections in both human and veterinary medicine, being also relevant to address the role of natural environments as important components of the dissemination cycle of these strains.

Regulations that cover the legal obligations that manufacturers are bound to are essential for keeping the general public safe. Companies need to follow the regulations in order to bring their products to market. A good understanding of the regulations and the regulatory pathway defines how fast and at what cost the manufacturer can introduce innovations to the market. Regulatory technology and data science can lead to new regulatory processes and evidence in the medical field. It can equip stakeholders with unique tools that can make regulatory decisions more objective, efficient, and accurate. This book describes the latest research within the broader domain of Medical Regulatory Technology (MedRegTech). It covers concepts such as the complexity and user-friendliness of medical device regulations, novel algorithms for regulatory navigation, descriptive datasets from a health service provider, regulatory data science techniques, and considerations of the environmental impacts within a national health service. This book brings all these aspects together to offer an introduction into MedRegTech research. In the long term, these technologies and methods will help optimize the regulatory strategy for individual healthcare innovations and revolutionize the way we engage with regulatory services.

Dear colleagues,This Special Issue, "Endothelial Dysfunction: From Pathophysiology to Novel Therapeutic Approaches", focuses on the pathophysiology of endothelial dysfunction, new biomarkers for endothelial dysfunction related to cardiovascular disorders or tumors, and novel therapeutic approaches for endothelial dysfunctions.Vascular endothelium is an active tissue and plays a crucial role in the maintenance of vascular homeostasis. Chronic exposure to risk factors, such as hypertension, high cholesterolemia, or oxidative stress, induces endothelial dysfunctions and results in a loss of endothelial integrity, smooth muscle cell proliferation, and macrophage recruitment.The pathophysiology of endothelial dysfunction (ED) is complex and multi-factorial factors are involved, such as oxidative stress or chronic inflammation. The primary prevention of cardiovascular risk factors and endothelial dysfunctions, as well as the early detection of or molecular imaging techniques for endothelial dysfunction, helps to prevent the development of cardiovascular disorders. Novel therapeutic approaches or drug delivery systems for endothelial dysfunctions have had promising beneficial effects in preclinical or clinical levels by affecting the progression of atherosclerotic changes, tumor angiogenesis, and host-immune reactions near tumor environments.

At present, cyanobacteria and their toxins (also known as cyanotoxins) constitute a major threat for freshwater resources worldwide. Cyanotoxin occurrence in water bodies around the globe is constantly increasing, whereas emerging, less studied or completely new variants and congeners of various chemical classes of cyanotoxins, as well as their degradation/transformation products are often detected. In addition to planctic cyanobacteria, benthic cyanobacteria, in many cases, appear to be important toxin producers, although far less studied and more difficult to manage and control. This Special Issue highlights novel research results on the structural diversity of cyanotoxins from planktic and benthic cyanobacteria, as well as on their expanding global geographical spread in freshwaters.

The Special Issue presents valuable information on the perspective of using cereals/pseudocereal grains, vegetable byproducts, or flour processed by applying various techniques in baked goods and pasta products. At the same time, this Special Issue reveals the experience of authors regarding the use of plants' ingredients as a valuable strategy to improve the nutritional profile of food products, contributing to the reduction in nutrient deficiencies, or to make products for special nutrition such as low-sodium or gluten-free ones. The quality of the raw materials, the processing techniques, and the potential of certain vegetable byproducts from fruits or oilseeds represent key aspects for obtaining qualitatively enhanced food products. Furthermore, current approaches to diversify the range of bakery and pasta products which offer health benefits to consumers are also presented in this Special Issue.

Carotenoids are uniquely functional polyene pigments that are ubiquitous in nature; aside from being responsible for the color of a wide variety of vegetables, interest has been focused on food carotenoids due to their likely health benefits.This Special Issue, "Carotenoids in Fresh and Processed Food: Between Biosynthesis and Degradation", consists of five peer-reviewed papers that cover a numerous new insights on the chemistry of carotenoids together with some observations related to their protection from photodegradation. Moreover, other considerations about their biosynthesis and influencing parameters in fresh food are included.