bluuebunny/crossref_metadata_2025_split huggingface dataset:
Splicing Modulation as Therapy for Human Genetic Disease
Authors: Srirangalingam Umasuthan , Khoo Bernard 
Published: July 2015

Abstract

The sequencing of the human genome has led to a greater appreciation of the contribution that genetic abnormalities and splicing errors make to the burden of human disease. The modulation of splicing using antisense oligonucleotides provides an attractive therapeutic option for tailored interventions in rare genetic disease. In this review, we outline the various approaches to splicing modulation and provide examples of each strategy. Finally, we address some of the practical issues facing the field in trying to bring this technology towards clinical application using the treatment of Duchenne muscular dystrophy and spinal muscular atrophy as examples.

Key Concepts

Ninety‐five percent of human genes undergo splicing.

An estimated 50% of all human genetic diseases result from splicing abnormalities.

Splicing modulation using antisense oligonucleotide (ASO) technology offers the future prospect of personalised medicine tailored to specific mutations.

Strategies to modulate splicing include suppression of an aberrant splice isoform, switching between known splicing variants or generating a novel variant with a potential therapeutic attribute.

ASO chemistries are varied and have been optimised to improve target binding and resist nuclease degradation.

ASO targets include all pre‐mRNA sites which influence the splicing process.

Splicing events can be complex and unpredictable. Therefore an iterative method of design and test is required to optimise ASO efficacy.

ASO side‐effects are likely to stem from the ASO chemistry, off‐target effects as the result of nonspecific binding, and ASO distribution to organs when given in vivo.

Optimising tissue delivery of ASO remains a significant challenge.
Aneuploidy and Protein Homeostatic Imbalance
Authors: Tang Yun‐Chi 
Published: July 2015

Abstract

Aneuploidy is an aberrant condition that a cell harbours incorrect chromosome numbers or identities often resulted from mitotic errors. Aneuploidy impacts cellular functions, developmental programme and affects individual fitness, and leads to examples such as Down syndrome and tumours. The gain or loss of large amount of genes caused by chromosome copy‐number changes can alter metabolic programmes and protein homeostasis. In most circumstances, the elicited stress responses which are detrimental to cellular and organismal fitness. However, in cancer cells, same stresses may be beneficial to pose adaptive potentials. In this article, the paradox of aneuploidy is discussed, and the triggering of aneuploidy‐associated protein homeostatic imbalance which may serve as a strategy to treat cancer.

Key Concepts

Aneuploidy is a situation that a cell harbours aberrant number of chromosomes that is not a multiple of the haploid complement.

Aneuploidy is mostly caused by mitotic errors and is irreversible in cells.

Aneuploidy generally resulted in adverse effect in both cellular and organismal levels; however, in stress conditions it may facilitate adaptation.

Aneuploidy causes protein homeostatic imbalance; thus, the proteotoxic property in cancer cells may apply as treatment for tumours.
Blogging, Political
Authors: Vaccari Cristian 
Published: July 2015

Abstract
Political blogs are online journals published by individuals, groups, and organizations to communicate, and usually facilitate discussion on, their political viewpoints. Blogs' impact on political communication is relevant in at least two domains: the flow of political information and the promotion and organization of political action. Political blogs are an integral part of contemporary hybrid media systems and share many functions and affordances with social media, although they maintain their distinctive features as a specific genre.
Introduction: Clean Energy Conversion Technologies
Authors: Desideri Umberto , Yan Jinyue 
Published: July 2015

Abstract
The power plants that have provided energy in the last century are facing a difficult future as renewable energy sources are taking a higher and higher share of the power generation. This is more evident in some areas of Europe, but the trend is towards a significant increase of renewable energy in the near future.
Conventional power plants, such as vapor cycles, gas turbines and internal combustion engines, which have powered our lives in the last 150 years, are now required new performance and environmental goals: high efficiency, flexibility and low or zero emissions. The research and development in the last 60 years have provided several solutions to improve the performance of those power plants. There are clear directions to follow to achieve low or zero emissions, but knowledge is not enough to reach the goals. Therefore, there is still a great need to find new and innovative solutions that will be a breakthrough towards the clean energy systems of the future.
This volume presents a number of energy conversion processes and systems which are currently being used in very large numbers, their development to the current state of art, the efforts that are still necessary to make this systems really “clean”, in order to stimulate the readers, either researchers or students, in using their brain to improve them in order to have “clean”, available and reliable systems for power generation.
Homogeneous Combustion Catalysts for Efficiency Improvements and Emission Reduction in Diesel Engines
Authors: Zhang Dongke , Zhu Mingming , Ma Yu , Millin Noel 
Published: July 2015

AbstractThis article presents an innovative catalytic technology of the use of ferrous‐picrate‐based homogeneous combustion catalysts that can be easily applied to improve fuel efficiency and reduce pollutant emissions from diesel engines. The homogeneous combustion catalysts, also referred to asfuel performance catalysts(FPC), can be dosed into the diesel at merely trace levels and are capable of improving fuel efficiency and reducing the emissions of smoke, unburned hydrocarbons, and carbon monoxide from diesel engines, which is supported by numerous laboratory diesel engine tests and field trials. The use of the catalysts enhances the fuel combustion rate and leads to more complete combustion and smaller sizes of primary soot particles and aggregates, which have lower ignition temperature and higher oxidative reaction rates. The catalysts are also capable of improving the fuel efficiency of biodiesel combustion in diesel engines. A sample case study of the economic and environmental benefits of this catalytic technology is also presented at the end of this article to illustrate the very significant benefits to be gained should the catalyst be widely adopted.
Smart Grid Impact on Operation and Planning of Electric Energy Systems
Authors: Su Wencong , Wang Jianhui , Ton Dan 
Published: July 2015

AbstractThe next‐generation electric power grid, also known as the Smart Grid, is expected to address the operational challenges of the nation's aging and outmoded electrical grid infrastructure. This article investigates the impacts of the Smart Grid technologies on operations and planning of electric energy systems at both the transmission and the distribution levels. This article presents a comprehensive overview of the emerging technologies (e.g., plug‐in electric vehicles, microgrids, distributed renewable energy sources and storage, demand‐side management and smart metering) and the development of distribution automation in Smart Grid. This article also discusses the technical information regarding the emerging technologies (e.g., wide‐area monitoring and measurements) and developments for transmission system operations in Smart Grid. There is still a significant amount of investigation to be done before the full vision of Smart Grid comes to fruition. Therefore, this article lists the future research needs covering both transmission and distribution systems in a Smart Grid environment.
Gasification and Combustion of Biomass: Physical Description and Mathematical Modeling
Authors: Fatehi Hesameddin , Bai Xue‐Song 
Published: July 2015

AbstractThe combustion and gasification of biomass are the major approaches to the utilization of biomass energy. This article discusses the fundamental processes involved in the gasification and combustion of biomass. The focus is on the mathematical description of the various subprocesses encountered in these thermochemical conversion processes, including drying, devolatilization/pyrolysis, char oxidation and gasification, shrinking of biomass, and heat and mass transfer inside the biomass particles. Phenomenological models for the various subprocesses are reviewed for numerical simulations of the thermochemical conversion process of the biomass particles. The models are evaluated by comparing the model results with experimental data under various experimental conditions. The basic thermodynamic properties of biomass, char, tar, and volatile gases are examined. It is shown that due to the large diversity of biomass characteristics, the measurement data out of experiments conducted in different facilities show a large scatter. Corresponding to this large scatter in experimental data, one can notice that there is a large scatter in model approaches, as well as the thermodynamic and chemical kinetic data reported in the literature. Phenomenological models can simulate the various experiments, provided that certain adjustments in the chemical kinetic data have been made. The performance of various submodels and suggested model parameters is demonstrated for various test cases.
Biomass Cofiring for Emissions Management
Authors: Tillman David A. 
Published: July 2015

AbstractBiomass cofiring in coal‐fired utility boilers has been demonstrated as an effective means to trim certain gaseous products of combustion including sulfur dioxide (SO2), fossil‐based carbon dioxide (CO2), and, for the most part, oxides of nitrogen (NOx). In some projects, it has also been shown to reduce mercury (Hg) emissions. As this article elucidates, the mechanisms by which biomass cofiring achieves these results include fuel composition—biomass is deficient in sulfur and its carbon is within the global carbon inventory available for recycling through the photosynthetic process. These mechanisms, when applied to NOxemissions reduction, capitalize on the high reactivity of the biomass fuel and the ability of reactive solid fuels to impart that reactivity to less reactive coals. The biomass fuels used in the United States are largely woody and herbaceous materials. Processed biomass in the form of pellets and/or torrefied wood has not been used extensively in the United States because of the high costs associated with double handling of the material and the processing itself. Animal manure has been tried but largely unsuccessfully. There have been numerous demonstrations and tests of cofiring; several such case studies are summarized in this article. These case studies document how the fuel properties of biomass are used in cofiring applications to achieve environmental benefits.
Photovoltaics: Building‐Integrated Photovoltaics
Authors: Balfour John R. 
Published: July 2015

Abstract

Building‐integrated photovoltaic (
BIPV
) technology is contributing to the growth and maturity of the photovoltaic (
PV
) industry by providing multi‐faceted construction products/components that generate energy while replacing building components. These projects differ from building‐attached photovoltaic (
BAPV
) in that
BIPV
is integrated into a structure and must be capable of replacement with a non‐
BIPV
component, whereas
BAPV
is
PV
attached mechanically to a building or structure.
BIPV
is a mix of energy generation, art, and architecture. Good
BIPV
is made to look simple, elegant, or stealthy. However, successful
BIPV
projects require mastering
PV
technology and its application, along with substantial
PV
and architectural knowledge, skill, education, and experience. Project effectiveness is produced by adopting a systems process where the
BIPV
is treated as a complete operational system within the building structure and not just the cobbling of products to meet local or national code. This article addresses the definitions, concepts, and considerations required to deliver effective and successful projects targeted with a 35–50‐year usable life while meeting the well‐defined goals and objectives set at the very beginning of the process.
BIPV
is not simple or easy. It can be a highly productive technology with subtle elegance.
Geothermal Fluids in Porous Media
Authors: Li Kewen , Bian Huiyuan , Cheng Changhui 
Published: July 2015

AbstractGeothermal energy is clean and renewable. It is important to understand the fundamental mechanisms and basic approaches to measure the parameters of fluids (steam and water) flowing in porous media. In this article, the techniques for measuring slip factor of steam, steam‐water relative permeability, and steam‐water capillary pressure were addressed. Also included were the mathematical models to calculate these parameters.
Internal Combustion Engine (ICE) Fundamentals
Authors: Grimaldi Carlo N. , Millo Federico 
Published: July 2015

AbstractThis is an introductory article, the purpose of which is to provide fundamental information on internal combustion engines (ICEs).In Section 1, the different types of ICEs are presented, and their role in the framework of the energy conversion systems is discussed.The morphology and the basic principles of operation are also described and discussed, along with the different possible classification criteria. The main performance parameters, commonly used for the comparative evaluation of different engines, are also presented and examined.Particular attention is given to the analysis of the ideal cycles to be considered as reference for the actual engines, while the different processes that represent the sources of the losses affecting the engine efficiency are evaluated. The basic principles that underlie the major efforts aimed to the development of new technologies for the reduction of the pollutant andCO2emissions are also presented.A section is then dedicated to the presentation and discussion of the legislation framework for the emissions generated by the ICEs, more recently extended from the standard pollutants toCO2emissions.The last section is then dedicated to the presentation of the typical engine performance maps and of the fundamental criteria useful for matching with the requirements of a specific application.
Low Carbon Economy inChina
Authors: Xue Jinjun , Bai Quan 
Published: July 2015

AbstractChina is the second largest economy, the largest trading country, and the largest carbon emitter and the largest energy consumption country in the world. As the most populous developing country at up‐middle income, economic growth will continually be the primary task of China, by this meaning, China will consumes more energy and emit moreCO2in the next decades. On the other hand, China has been promoting a Green‐Low‐Carbon Development Strategy and making great efforts in energy conservation and emission reduction according to its promised emission reduction target of 40–45% per unitGDP(gross domestic product ) by 2020 and the targets in the 12th five‐year plan. This article presents China's economic growth, introduces its climate change and energy policy, valuates its efforts to energy conservation and emission reduction, analyzes the problems and challenges, and, most importantly, projects China's turning points of energy consumption and carbon emissions by years 2020 and 2050.
Thermal Energy Efficiency in Industrial Processes
Authors: Rajendran Keerthi , Ling‐Chin Janie , Roskilly Anthony P. 
Published: July 2015

Abstract
Thermal energy used for heating and cooling represents a significant proportion of total energy use in industrial processes and is largely supplied by fossil fuels. Mechanical operations primarily powered by electricity also consume a large amount of energy. Both heating and cooling operations as well as mechanical operations release large amounts of heat into the environment; therefore, careful management of thermal energy is a key factor in reducing overall energy consumption and emissions.
Energy‐related pressures, incentives, and opportunities provide a strong business case to invest in improving energy efficiency. These include customer demand for “green” products, national taxes, and financial incentives, and rising fuel costs. Against this backdrop, the prioritization of the sustainability agenda by research funders coupled with the pressure on research organizations to ensure the transfer of novel technologies to industry creates an ideal landscape for substantial advancement in industrial energy efficiency. Successful movement of novel technology to industry should, however, be supported by concepts, methods, and practices derived from industrial context and needs. Accordingly, initial implementations often bring to light new research challenges, both technical and methodological.
To enhance the use of energy‐efficient methods in industry, the definition and measurement of efficiency must be tailored to industrial needs and technologies ready for adoption must be identified and presented in relation to existing technology. In addition, detailed case studies of energy use should be documented to provide insights to both researchers and practitioners, identifying both realistic improvements and barriers to application of energy‐efficient measures.
Emerging Technologies for Waste‐to‐Energy and Fuel Conversion
Authors: Yoshikawa Kunio 
Published: July 2015

Abstract
Up to now, the only solution for utilization of solid wastes including biomass as energy resources is to incinerate them (mass burning) and to use the produced heat directly or to convert the produced heat into electric power employing boilers/steam turbines. This process should be large scale and expensive to install and operate. In this article, new technologies for converting unutilized resources such as solid wastes and biomass into high value added energy resources (solid fuel, gaseous fuel, liquid fuel, and electric power) with small to medium scale are introduced for promoting distributed waste utilization. The content of the technologies are as follows. Solid fuel production technology: the hydrothermal treatment technology can convert unutilized resources with various shapes, heating values, and moisture contents into uniform dry powder‐like solid fuels with the heating value equivalent to coal. Liquid fuel production technology: diesel equivalent fuel oils can be produced from waste plastics by employing the pyrolytic reforming oil production technology and from waste cooking oils by employing the dry‐type alkali catalyst biodiesel fuel production technology. The emulsion fuel that is a mixture of oil and water can reduce the emissions as well as improve the thermal efficiency of boilers. Gasification and power generation technology: the gasification technologies can produce low to medium calorific gases from solid wastes by employing pyrolysis and reforming or steam gasification technologies. Electric power can be generated from this low to medium calorific gases by employing internal combustion engines.
Waste and Biomass to Clean Energy
Authors: Gupta Ashwani K. 
Published: July 2015

AbstractWaste and biomass include different kinds of organic and inorganic materials including wood, trees, agricultural, municipal, medical, hazardous, electronic, industrial solid wastes, and waste glass, metals, and plastics. Waste is a growing problem to the society worldwide. Increased waste production is directly related to increased gross domestic product (GDP) so that one must seek options for their safe and environmentally benign disposal. The old practice of landfills, although still in use in many countries, is less acceptable as this causes odors and nuisance to the nearby community and can be leachable to ground water. Thermal destruction and incineration is now widely used as it provides energy recovery (both thermal and electricity), mass and volume reduction of the waste, and the remaining residue can be used for construction material or discarded at landfills. Pyrolysis and gasification offers clean reforming of wastes to syngas, which can also be converted into liquid fuels. Much of the work on gasification of wastes is at the laboratory scale so that the favorable results obtained can be used at pilot‐ and demonstration‐scale plants, akin to coal gasification plants. Waste and biomass can also be converted into useful clean refuse‐derived fuel and value added products of activated carbon and torrefied material. It is expected that the fundamental information on pyrolysis and gasification provided here will assist in the design and operation of advanced gasifiers with eliminated tars and char. The reforming of wastes to useful materials and value added products provide clean energy to support energy sustainability.
Pumped Hydro Energy Storage
Authors: Deane Paul , Gallachóir Brian Ó 
Published: July 2015

AbstractPumped hydroelectric storage is currently the only commercially proven large‐scale (>100 MW) energy storage technology with over 200 plants installed worldwide with a total installed capacity of over 100 GW. The fundamental principle of pumped hydroelectric storage is to store electric energy in the form of hydraulic potential energy. Pumping typically takes place during off‐peak periods, when both electricity demand and electricity prices are low. Generation takes place during peak periods, when electricity system demand is high. Pumping and generating generally follow a daily cycle but weekly or even seasonal cycling is also possible with larger pumped hydroelectric storage plant. Pumped hydroelectric storage is a flexible form of electricity generation and can contribute many benefits to power systems operation. There has been a renewed commercial and technical interest in pumped hydroelectric storage recently with the advent of increased variable renewable energy generation and the development of liberalized electricity markets.
Methods for Measuring and Evaluating Sustainability: State‐of‐the Art, Challenges, and Future Developments
Authors: Kleeberg Kirstin , Schneider Katja , Nippa Michael 
Published: July 2015

AbstractBy now, the termsustainabilitysuffers from being degenerated into a buzzword resulting in an exceptionally large amount and variety of literature. Likely due to this, the dispersal of descriptions, perspectives, and facets regarding sustainability‐assessing methods reveals deficits in terms of theoretical and methodological rigor, novelty, and practical usefulness. Against this background, this article contains an overview of eight selected methods and tools for sustainability assessment. The qualitative review of current research articles concerning energy technologies highlights objectives and purposes, origins, main procedures, fields of application, and examples as well as a critical acclaim for each method. Next to this, several considerations in general such as the role of principles, criteria, and indicators are being illustrated as well as important challenges for measuring sustainability. Thereby, we develop a six‐stage model that supports the choice of an appropriate method for sustainability assessment. In doing so, we derive two fundamental principles, namely “content‐related” features and “performance‐related” features. At the same time, this article underlines the coexistence of various different, highly complex methods, approaches, and perspectives, as well as practical examples to assess sustainability. We evidence an increasing defragmentation and specialization of research on sustainability assessment. Against this background, our findings are likely to foster further theoretical, conceptual, and analytical developments in this research field. This also promotes a customization and the practical value of sustainability assessment methods.