A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeld-Jakob disease (vCJD)

The objective of this study was to determine the degree of white matter pathology in the cerebral cortex in cases of variant Creutzfeldt-Jakob disease (vCJD) and to study the relationships between the white matter and grey matter pathologies. Hence, the pathological changes in cortical white matter were studied in individual gyri of the frontal, parietal, occipital, and temporal cortex in eleven cases of vCJD. Vacuolation (‘spongiform change’), deposition of the disease form of prion protein (PrPsc) in the form of discrete PrP deposits, and gliosis were observed in the white matter of virtually all cortical regions studied. Mean density of the vacuoles in the white matter was greater in the parietal lobe compared with the frontal, occipital, and temporal lobes but there were fewer glial cells in the occipital lobe compared with the other cortical regions. In the white matter of the frontal cortex, vacuole density was negatively correlated with the density of both glial cell nuclei and the PrP deposits. In addition, the densities of glial cells and PrP deposits were positively correlated in the frontal and parietal cortex. In the white matter of the frontal cortex and inferior temporal gyrus, there was a negative correlation between the densities of the vacuoles and the number of surviving neurons in laminae V/VI of the adjacent grey matter. In addition, in the frontal cortex, vacuole density in the white matter was negatively correlated with the density of the diffuse PrP deposits in laminae II/III and V/VI of the adjacent grey matter. The densities of PrP deposits in the white matter of the frontal cortex were positively correlated with the density of the diffuse PrP deposits in laminae II/III and V/V1 and with the number of surviving neurons in laminae V/V1. The data suggest that in the white matter in vCJD, gliosis is associated with the development of PrP deposits while the appearance of the vacuolation is a later development. In addition, neuronal loss and PrP deposition in the lower cortical laminae of the grey matter may be a consequence of axonal degeneration within the white matter.

Neuropathology of Basal Ganglia and its role in the Parkinsonian syndromes with special reference to the Globus pallidus

The globus pallidus, together with the striatum (caudate nucleus and putamen), substantia nigra, nucleus accumbens, and subthalamic nucleus constitute the basal ganglia, a group of nuclei which act as a single functional unit. The basal ganglia have extensive connections to the cerebral cortex and thalamus and exert control over a variety of functions including voluntary motor control, procedural learning, and motivation. The action of the globus pallidus is primarily inhibitory and balances the excitatory influence of other areas of the brain such as the cerebral cortex and cerebellum. Neuropathological changes affecting the basal ganglia play a significant role in the clinical signs and symptoms observed in the ‘parkinsonian syndromes’ viz., Parkinson’s disease (PD), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB), multiple system atrophy (MSA), and corticobasal degeneration (CBD). There is increasing evidence that different regions of the basal ganglia are differentially affected in these disorders. Hence, in all parkinsonian disorders and especially PD, there is significant pathology affecting the substantia nigra and its dopamine projection to the striatum. However, in PSP and MSA, the globus pallidus is also frequently affected while in DLB and CBD, whereas the caudate nucleus and/or putamen are affected, the globus pallidus is often spared. This chapter reviews the functional pathways of the basal ganglia, with special reference to the globus pallidus, and the role that differential pathology in these regions may play in the movement disorders characteristic of the parkinsonian syndromes.

Cognitive systems associated with the hippocampus of the human brain and their role in behaviour and neurodegenerative disease

Cognitive systems research involves the synthesis of ideas from natural and artificial systems in the analysis, understanding, and design of all intelligent systems. This chapter discusses the cognitive systems associated with the hippocampus (HC) of the human brain and their possible role in behaviour and neurodegenerative disease. The hippocampus (HC) is concerned with the analysis of highly abstract data derived from all sensory systems but its specific role remains controversial. Hence, there have been three major theories concerning its function, viz., the memory theory, the spatial theory, and the behavioral inhibition theory. The memory theory has its origin in the surgical destruction of the HC, which results in severe anterograde and partial retrograde amnesia. The spatial theory has its origin in the observation that neurons in the HC of animals show activity related to their location within the environment. By contrast, the behavioral inhibition theory suggests that the HC acts as a ‘comparator’, i.e., it compares current sensory events with expected or predicted events. If a set of expectations continues to be verified then no alteration of behavior occurs. If, however, a ‘mismatch’ is detected then the HC intervenes by initiating appropriate action by active inhibition of current motor programs and initiation of new data gathering. Understanding the cognitive systems of the hippocampus in humans may aid in the design of intelligent systems involved in spatial mapping, memory, and decision making. In addition, this information may lead to a greater understanding of the course of clinical dementia in the various neurodegenerative diseases in which there is significant damage to the HC.

Pathology of the dentate gyrus in neurodegenerative disease

The dentate gyrus (DG) is an important part of the hippocampal formation and is believed to be involved in a variety of brain functions including episodic and spatial memory and the exploration of novel environments. In several neurodegenerative disorders, significant pathology occurs in the DG which may be involved in the development of clinical dementia. Based on the abundance of pathological change, neurodegenerative disorders can be divided into three groups: (1) those in which high densities of neuronal cytoplasmic inclusions (NCI) are present in DG granule cells, e.g., Pick’s disease (PiD), frontotemporal lobar degeneration with TDP-43-immunoreactive inclusions (FTLD-TDP), and neuronal intermediate filament inclusion disease (NIFID), (2) those in which aggregated protein deposits are distributed throughout the hippocampal formation including the molecular layer of the DG, e.g., Alzheimer’s disease (AD), Down’s syndrome (DS), and variant Creutzfeldt-Jakob disease (vCJD), and (3) those in which in there is significantly less pathology in the DG, e.g., Parkinson’s disease dementia (PD-Dem), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA), and sporadic CJD (sCJD). Hence, DG pathology varies significantly among disorders which could contribute to differences in clinical dementia. Pathological differences among disorders could reflect either differential vulnerability of the DG to specific molecular pathologies or variation in the degree of spread of pathological proteins into the hippocampal formation from adjacent regions.

Preface

The Semantic Web has come a long way since its inception in 2001, especially in terms of technical development and research progress. However, adoption by non- technical practitioners is still an ongoing process, and in some areas this process is just now starting. Emergency response is an area where reliability and timeliness of information and technologies is of essence. Therefore it is quite natural that more widespread adoption in this area has not been seen until now, when Semantic Web technologies are mature enough to support the high requirements of the application area. Nevertheless, to leverage the full potential of Semantic Web research results for this application area, there is need for an arena where practitioners and researchers can meet and exchange ideas and results. Our intention is for this workshop, and hopefully coming workshops in the same series, to be such an arena for discussion. The Extended Semantic Web Conference (ESWC - formerly the European Semantic Web conference) is one of the major research conferences in the Semantic Web field, whereas this is a suitable location for this workshop in order to discuss the application of Semantic Web technology to our specific area of applications. Hence, we chose to arrange our first SMILE workshop at ESWC 2013. However, this workshop does not focus solely on semantic technologies for emergency response, but rather Semantic Web technologies in combination with technologies and principles for what is sometimes called the "social web". Social media has already been used successfully in many cases, as a tool for supporting emergency response. The aim of this workshop is therefore to take this to the next level and answer questions like: "how can we make sense of, and furthermore make use of, all the data that is produced by different kinds of social media platforms in an emergency situation?" For the first edition of this workshop the chairs collected the following main topics of interest: • Semantic Annotation for understanding the content and context of social media streams. • Integration of Social Media with Linked Data. • Interactive Interfaces and visual analytics methodologies for managing multiple large-scale, dynamic, evolving datasets. • Stream reasoning and event detection. • Social Data Mining. • Collaborative tools and services for Citizens, Organisations, Communities. • Privacy, ethics, trustworthiness and legal issues in the Social Semantic Web. • Use case analysis, with specific interest for use cases that involve the application of Social Media and Linked Data methodologies in real-life scenarios. All of these, applied in the context of: • Crisis and Disaster Management • Emergency Response • Security and Citizen Journalism The workshop received 6 high-quality paper submissions and based on a thorough review process, thanks to our program committee, the decision was made to accept four of these papers for the workshop (67% acceptance rate). These four papers can be found later in this proceedings volume. Three out of four of these papers particularly discuss the integration and analysis of social media data, using Semantic Web technologies, e.g. for detecting complex events in social media streams, for visualizing and analysing sentiments with respect to certain topics in social media, or for detecting small-scale incidents entirely through the use of social media information. Finally, the fourth paper presents an architecture for using Semantic Web technologies in resource management during a disaster. Additionally, the workshop featured an invited keynote speech by Dr. Tomi Kauppinen from Aalto university. Dr. Kauppinen shared experiences from his work on applying Semantic Web technologies to application fields such as geoinformatics and scientific research, i.e. so-called Linked Science, but also recent ideas and applications in the emergency response field. His input was also highly valuable for the roadmapping discussion, which was held at the end of the workshop. A separate summary of the roadmapping session can be found at the end of these proceedings. Finally, we would like to thank our invited speaker Dr. Tomi Kauppinen, all our program committee members, as well as the workshop chair of ESWC2013, Johanna Völker (University of Mannheim), for helping us to make this first SMILE workshop a highly interesting and successful event!

Oligodendrocyte pathology in neurodegenerative disease

Oligodendrocytes have multiple functions in the central nervous system including mechanical support of neurons, production of myelin sheaths, and uptake and inactivation of chemical neurotransmitters released by neurons. Consequently, oligodendrocytes could be involved in the pathology of a number of neurodegenerative diseases. Although, the molecular mechanisms involved require further elucidation, it is likely that oligodendrocyte dysfunction is important in Alzheimer’s disease (AD), amyotrophic lateral sclerosis (ALS), and multiple sclerosis (MS). In addition, abnormal protein aggregates in the form of oligodendrocyte inclusions (OI) have been observed in several other disorders, most notable in multiple system atrophy (MSA), in which the glial cytoplasmic inclusion (GCI) is the ‘signature’ pathology of the disease. OI have also been identified in argyrophilic grain disease (AGD), progressive supranuclear palsy (PSP) (Armstrong et al 2007), and various forms of frontotemporal lobar degeneration (FTLD) (Armstrong et al 2010), although their role in the pathology of these disorders is less clear. It is likely that future research will expand the range of disorders in which oligodendrocytes play a significant role in neurodegeneration.

Biochemical Characterization of Human Exonuclease 1 Protein-protein Interactions

Human Exonuclease 1 (Exo1) plays important roles in numerous DNA metabolic/repair pathways including DNA mismatch repair, DNA double strand break repair, Okazaki fragment maturation etc. The nuclease activity of Exo1 is tightly regulated in vivo. The regulation of Exo1 in different pathways is achieved by interactions with different protein partners. The focus of this dissertation will be on characterization of Exo1 interactions with traditional protein partners and providing experimental evidences for new Exo1 interactions.

Molecular cloning, biochemical assays, collaborative nuclear magnetic resonance and X-ray crystallography have been employed to study Exo1 interactions with protein partners. This work contains: (i) the experimental evidence for new Exo1 interactions, and (ii) the detailed characterization of Exo1 interactions with PCNA, MLH1 and MutSα/β.

Taken together, the research progress presented in this dissertation further advances our understanding of traditional Exo1 interaction network and probably provides new insights to new functions and new regulations of Exo1.

Table 2. Performance summary of K-based sorbents capturing CO2

This article reviews the progress made in CO2 capture, storage, and utilization in Chinese Academy of Sciences (CAS). New concepts such as adsorption using dry regenerable solid sorbents as well as functional ionic liquids (ILs) for CO2 capture are thoroughly discussed. Carbon sequestration, such as geological sequestration, mineral carbonation and ocean storage are also covered. The utilization of CO2 as a raw material in the synthesis of chemicals and liquid energy carriers which offers a way to mitigate the increasing CO2 buildup is introduced.

How to progress forward in improving safety outcomes in the work-related driving field : a review of work-related driving research and development of an intervention framework [Extended abstract]

Road crashes are now the most common cause of work-related injury, death and absence in a number of countries. Given the impact of workrelated driving crashes on social and economic aspects of business and the community, workrelated road safety and risk management has received increasing attention in recent years. However, limited academic research has progressed on improving safety within the work-related driving sector. The aim of this paper is to present a review of work-related driving safety research to date, and provide an intervention framework for the future development and implementation of workrelated driving safety intervention strategies.

Assessing information systems success models : empirical comparison (Research in Progress)

Information System (IS) success may be the most arguable and important dependent variable in the IS field. The purpose of the present study is to address IS success by empirically assess and compare DeLone and McLean’s (1992) and Gable’s et al. (2008) models of IS success in Australian Universities context. The two models have some commonalities and several important distinctions. Both models integrate and interrelate multiple dimensions of IS success. Hence, it would be useful to compare the models to see which is superior; as it is not clear how IS researchers should respond to this controversy.

Towards an orchestration theory in business transformation management (research in progress)

Business transformations are large-scale organisational change projects that, evidence suggests, are often unsuccessful. This study aims to develop a conceptual model that explains how management services that are required for a business transformation are orchestrated during such a initiative. We classify management services such as (but not limited to) change management, risk management, IT management, financial management, program management and so forth as bearing transformational and/or transactional capabilities in a transformation initiative. We then draw upon three principles of musical composition, namely melody, harmony and rhythm, and illustrate how they apply to the orchestration of management services in the management of business transformations. In order to illustrate our emerging model, we examine the case of Malaysia Airlines, who have managed to successfully turnaround the near-bankrupt organisation beyond survival. We demonstrate how the notions of melody, harmony and rhythm can be used to describe their endeavour. We conclude by discussing next steps of our research.