Miracle-dz/UniFAR-FTUs huggingface dataset:
Phenotypic variety in the presentation of frontotemporal lobar degeneration

Abstract Frontotemporal lobar degeneration (FTLD) is a heterogeneous syndrome characterized by a progressive decline in behaviour or language associated with degeneration of the frontal and anterior temporal lobes. FTLD can be classified into three clinical syndromes based on the early and predominant symptoms: behavioural variant frontotemporal dementia (bvFTD); semantic dementia (SD); and progressive non-fluent aphasia (PNFA). Patients with bvFTD present with marked changes in personality and behaviour such as disinhibition, apathy, loss of sympathy, compulsive behaviours, hyperorality. Neuroimaging studies have highlighted frontal atrophy, hypometabolism and hypoperfusion. SD is characterized by a fluent anomic aphasia and behavioural changes with degeneration of the anterior temporal lobes. Patients with left-sided SD present with progressive loss of word knowledge and meaning about words, objects and concepts. Patients with PNFA present slow, effortful speech, impaired production and comprehension of grammar. PNFA is associated with atrophy, hypometabolism and hypoperfusion of the left perisylvian area. However, overlap between the syndromes can occur, particularly later in the course. In the absence of definitive biomarkers, the diagnosis is dependent on clinical symptoms. A new diagnostic criterion of bvFTD was published in 2011. Identification of the syndrome's core symptoms is important in clinical diagnosis.
The origins of the aesthetic enjoyment of music — A review of the literature

Listeners attribute a positive or negative value to music. This aesthetic experience is known to be observable over the individuals’ entire life span, from early childhood to old age, and in every culture. It is then often concluded that such an aesthetic experience constitutes part of the human nature, having an important biological foundation. This assumption leads to the question of how aesthetic experience of music originated in the biological evolution. In this paper, we will explore this question in the light of evolutionary psychology, cognitive science, and systems neuroscience. After reviewing the existing proposals, we conclude that the explanation of the origin of aesthetic experience in music is likely to involve a more heterogeneous set of biological mechanisms than what is often assumed.
Human Information Processing and Adjudication: Trial by Heuristics

This article addresses the role of quantitative evidence and methods in trials. Major arguments against the introduction of explicit computation information are considered and contrasted with findings about the characteristics of the unaided human decision maker. Emphasis is given to behavioral decision theory and the heuristic biases it reveals. Consideration is given to the symbolic versus 'truthfinding" functions of trials, mathematical models (especially linear) of decision making, advocacy tactics that follow from what has been learned about how humans process information, the diagnostic value of aggregate probabilities, the notion of particularistic proof, and the evaluation of witness credibility. We conclude that unaided human decision making embodies certain normal and lawful errors, and that the exclusion of mathematical guides to aid a fact finder, while avoiding some problems, exposes the fact-finding process to the heuristic biases of intuitive decision making.
Education as a Basic Human Right: A Response to Special Education and the Charter

“Special Education and the Charter: The Right to Equal Benefit of the Law” is an excellent article on the provincial statutory regimes and their relationship to s. 15 of the Charter. It surveys the legislatures' attempts at delivering education to students and highlights the shortcomings in these attempts, focussing on the inability or unwillingness of the legislatures to provide an appropriate education to mentally disabled individuals. The article then takes a prospective approach, illustrating how a generous interpretation of s. 15 of the Charter might be used to correct deficiencies in educational statutes. Both the survey of statute law and the commentary on its relationship to the equality provisions of the Charter provide a valuable addition to scholarly writing on the provision of appropriate education to the mentally disabled. However, there is some danger in assuming that the right to education derives solely from statute. Statutes are creations of legislatures. If the right to education exists only in these statutes, education may be viewed not as the right of every child, but as a privilege bestowed by the legislature, to be determined by administrators, and to be overseen only as a last resort by the courts.
An Empirical Validation of Object-Oriented Metrics in Two Different Iterative Software Processes

Object-oriented (OO) metrics are used mainly to predict software engineering activities/efforts such as maintenance effort, error proneness, and error rate. There have been discussions about the effectiveness of metrics in different contexts. In this paper, we present an empirical study of OO metrics in two iterative processes: the short-cycled agile process and the long-cycled framework evolution process. We find that OO metrics are effective in predicting design efforts and source lines of code added, changed, and deleted in the short-cycled agile process and ineffective in predicting the same aspects in the long-cycled framework process. This leads us to believe that OO metrics' predictive capability is limited to the design and implementation changes during the development iterations, not the long-term evolution of an established system in different releases.
Talking-Ally: What is the future of robot's utterance generation?

It is still an enormous challenge within the HRI community to make a significant contribution to the development of a robot's utterance generation mechanism. How does one actually go about contributing and predicting the future of robot utterance generation? Since, our motivation to propose a robot's utterance generation approach by utilizing both addressivity and hearership. Novel platform of Talking-Ally is capable of producing an utterance (toward addressivity) by utilizing the state of the hearer's behaviors (eye-gaze information) to persuade the user (states of hearership) through dynamic interaction. Moreover, the robot has the potential to manipulate modality, turn-initial, and entrust behaviors to increase the liveliness of conversations, which are facilitated by shifting the direction of the conversation and maintaining the hearer's engagement in the conversation. Our experiment focuses on evaluating how interactive users engage with an utterance generation approach (performance) and the persuasive power of robot's communication within dynamic interactions.
Implementing GliderAgent - an agent-based decision support system for glider pilots

The aim of this chapter is to discuss practical issues encountered when a skeleton of the GliderAgent—a multi-agent system supporting glider pilots in various navigation and pilotage scenarios—was implemented. The GliderAgent implementation integrates two agent environments discussed in other chapters of this book (Jade and MAPS), as well as the XCsoar pilotage software, and the OpenStreetMaps GIS system. In addition to presenting the basic design of the GliderAgent system, the technical issues solved during its implementation are discussed. Finally, examples of implemented capabilities are illustrated.
Human SWI/SNF Generates Abundant, Structurally Altered Dinucleosomes on Polynucleosomal Templates

ABSTRACT Human SWI/SNF (hSWI/SNF) is an evolutionarily conserved ATP-dependent chromatin remodeling complex required for transcriptional regulation and cell cycle control. The regulatory functions of hSWI/SNF are correlated with its ability to create a stable, altered form of chromatin that constrains fewer negative supercoils than normal. Our current studies indicate that this change in supercoiling is due to the conversion of up to one-half of the nucleosomes on polynucleosomal arrays into asymmetric structures, termed “altosomes,” each composed of two histone octamers and bearing an asymmetrically located region of nuclease-accessible DNA. Altosomes can be formed on chromatin containing the abundant mammalian linker histone H1 and have a unique micrococcal nuclease digestion footprint that allows their position and abundance on any DNA sequence to be measured. Over time, altosomes spontaneously revert to structurally normal but improperly positioned nucleosomes, suggesting a novel mechanism for transcriptional attenuation as well as transcriptional memory following hSWI/SNF action.
An Analysis Tool for UML Models with SPT Annotations

In this paper, we describe a plug-in for the Rhapsody tool, which demonstrates how UML models with SPT annotations can be analysed using the Times tool — a tool for modelling, schedulability analysis, and code generation for timed systems. The plug-in takes as input an UML model consisting of an assembly of components whose behaviours are specified by statecharts. Operations may be annotated with SPT timing parameters for their execution time, deadline, priority etc. The output is a network of timed automata extended with tasks that can be analysed using the Times tool. In particular, the Times tool will show whether the operations invoked from the UML model are guaranteed to meet their deadlines or not. We describe how this has been done in a case study where an SPT annotated UML model of an adaptive cruise controller is studied. 1 UML with SPT Annotations We use a subset of UML with SPT annotations (the UML profile for Scheduling, Performance and Time [11]) as an input language for our tool. The models that can be analysed are hierarchical static structure diagrams containing objects representing components or assemblies of components. The behaviour of each component is described by an associated statechart, each executing in its own thread of control. Figure 1 shows an example which can be analysed with the tool presented in this paper. Component A is composed of subcomponents B and C whose corresponding statecharts are shown to the right of the structure diagram. The operations of components represent tasks being scheduled for execution. We apply the SPT stereotype SAAction for operations to annotate them with parameters such as priority, computation time, deadline, etc. Parameters of the operation foo of C are shown in Figure 1. Operations are triggered by statecharts, and operations triggered by the same statechart are executed synchronously. Components can have ports that can be connected with directed links allowing for interaction between them. There are two types of ports, namely data ports and trigger ports. Data ports are defined using DataPort interface which has two methods get and set. Links between data ports are one place buffers that
Perception of fatigue and couple communication in people with multiple sclerosis and their spouses: relationship to coping efficacy

The purpose of t h i s e x p l o r a t o r y study was to determine the extent to which congruence i n the per c e p t i o n of f a t i g u e impact between people w i t h m u l t i p l e s c l e r o s i s (MS) and t h e i r spouses i s r e l a t e d to the coping e f f i c a c y of the i n d i v i d u a l s w i t h MS. Although f a t i g u e i s a w e l l known symptom i n MS, the nature of i t s impact on the i n d i v i d u a l w i t h the disease as w e l l as on t h e i r spouse i s p o o r l y understood. Working from Lazarus and Folkman's (1984) model of s t r e s s and coping, I questioned whether i n d i v i d u a l s w i t h MS appraise the impact of t h e i r f a t i g u e d i f f e r e n t l y than do t h e i r spouses, and whether the degree of incongruence i s r e l a t e d to the coping e f f i c a c y of the person w i t h MS. C r i t e r i a f o r i n c l u s i o n i n c l u d e d married couples i n which one person: (a) had been diagnosed w i t h MS f o r at l e a s t one year, and (b) i d e n t i f i e d f a t i g u e as a symptom. The ENRICH Couple Communication Scale (Olson, 1985) and The Fatigue Impact Scale (Fis k , P o n t e f r a c t , R i t v o , A r c h i b a l d , & Murray, 1994) were administered to 60 couples i n which one person had MS. I n d i v i d u a l s w i t h MS then r a t e d t h e i r p e r c e p t i o n of coping e f f i c a c y using a modified v e r s i o n of the Coping Strategy I n d i c a t o r (Amirkhan, 1990). E f f i c a c y of avoidance, problem-
Role of Inhibitory Functions in Working Memory: Evidence in Children and Adolescents

Objective. Different theoretical positions have posted a close relationship between working memory (WM) and inhibition. Therefore, the aim of this study was to analyze the role played by inhibitory processes or perceptual inhibition, cognitive inhibition and restraint inhibition in the development of WM between 8 and 1 Esta investigacion se financio parcialmente con el apoyo de la beca Doctoral Conicet de la primera autora (Proyecto: Comprension lectora y Procesos cognitivos) 2 Dra. en Psicologia. Correo de Correspondencia: canetjuric@mdp.edu. ar 3 Dra. en Psicologia 4 Lic. en Psicologia 5 Dra. en Psicologia LORENA CANET JURIC, MARIA LAURA ANDRES, SILVINA DEMAGISTRI, GRAZIELLA MASCARELLO Y DEBORA BURIN 110 18 years. Method. The sample consisted of 277 participants from the city of Mar del Plata divided into three age groups (8-9 years, 10-12 years and 17-18 years). Participants were assessed with a task of WM that provide several indicators of inhibitory functions. Results. Study results showed that performance in WM is higher in older participants, with significant differential effect of age on WM. Significant effects were observed in relation to the type of inhibition and the age group. Conclusion. Consistent with previous studies, a small relationship between WM perceptual inhibition and restraint inhibition, this relationship is mediated by age.
Analyzing the causes and spatial pattern of the European 2003 carbon flux anomaly using seven models

Abstract. Globally, the year 2003 is associated with one of the largest atmospheric CO2 rises on record. In the same year, Europe experienced an anomalously strong flux of CO2 from the land to the atmosphere associated with an exceptionally dry and hot summer in Western and Central Europe. In this study we analyze the magnitude of this carbon flux anomaly and key driving ecosystem processes using simulations of seven terrestrial ecosystem models of different complexity and types (process-oriented and diagnostic). We address the following questions: (1) how large were deviations in the net European carbon flux in 2003 relative to a short-term baseline (1998–2002) and to longer-term variations in annual fluxes (1980 to 2005), (2) which European regions exhibited the largest changes in carbon fluxes during the growing season 2003, and (3) which ecosystem processes controlled the carbon balance anomaly . In most models the prominence of 2003 anomaly in carbon fluxes declined with lengthening of the reference period from one year to 16 years. The 2003 anomaly for annual net carbon fluxes ranged between 0.35 and –0.63 Pg C for a reference period of one year and between 0.17 and –0.37 Pg C for a reference period of 16 years for the whole Europe. In Western and Central Europe, the anomaly in simulated net ecosystem productivity (NEP) over the growing season in 2003 was outside the 1σ variance bound of the carbon flux anomalies for 1980–2005 in all models. The estimated anomaly in net carbon flux ranged between –42 and –158 Tg C for Western Europe and between 24 and –129 Tg C for Central Europe depending on the model used. All models responded to a dipole pattern of the climate anomaly in 2003. In Western and Central Europe NEP was reduced due to heat and drought. In contrast, lower than normal temperatures and higher air humidity decreased NEP over Northeastern Europe. While models agree on the sign of changes in simulated NEP and gross primary productivity in 2003 over Western and Central Europe, models diverge in the estimates of anomalies in ecosystem respiration. Except for two process models which simulate respiration increase, most models simulated a decrease in ecosystem respiration in 2003. The diagnostic models showed a weaker decrease in ecosystem respiration than the process-oriented models. Based on the multi-model simulations we estimated the total carbon flux anomaly over the 2003 growing season in Europe to range between –0.02 and –0.27 Pg C relative to the net carbon flux in 1998–2002.
Low intensity strength training for ambulatory stroke patients

Purpose. To investigate feasibility and effectiveness of an individually-directed, group strength-training programme on knee muscle strength after stroke. Method. Ten volunteers (62 ± 11 years, mean ± SD), 6 – 12 months after first-ever unilateral stroke, walking independently with or without aids were recruited. Using an A1-B-A2 design, 3 sets of baseline measures were taken at 2 weekly intervals; volunteers then attended twice weekly sessions of low intensity progressive strengthening exercises and were assessed after each series of 8 sessions to a maximum of 24 sessions; post training, measures were repeated after 4 – 6 weeks. Measures included isometric and concentric knee extensor muscle strength and 10 m walking velocity. Results. Strength of knee extensor muscles was improved after training (ANOVA, p < 0.05). On cessation of training, isometric strength increased by 58 ± 19% and concentric strength at 30°/s by 51 ± 14%; walking velocity quickened from 0.47 ± 0.06 m · s−1 to 0.57 ± 0.08 m · s−1 (t = −3.31, p < 0.01). These gains were maintained 4 – 6 weeks after completion of training. Conclusions. These findings support the use of low intensity strength training after stroke and confirm published evidence. It was feasible for one therapist to deliver the training programmes for 4 – 6 participants at a time; an important feature when resources are limited.
Expression of proteins essential for IS1 transposition: specific binding of InsA to the ends of IS1.

The insertion sequence IS1 displays a complex array of open reading frames (ORF). In an attempt to identify those which encode polypeptide products, we have systematically placed each ORF under the control of the P1 promoter of phage lambda. In the expression system we used, only the product of the insA gene was present in high enough amounts to be detected by polyacrylamide gel electrophoresis. The production of InsA was further increased in a first codon hook‐up to phage T7 transcriptional and translational initiation signals. Cell extracts from InsA overproducers display a DNA binding activity specific for the ends of IS1. This activity was identified as the InsA protein itself.
Activating Mutations of the TRPML1 Channel Revealed by Proline-scanning Mutagenesis*

The mucolipin TRP (TRPML) proteins are a family of endolysosomal cation channels with genetically established importance in humans and rodent. Mutations of human TRPML1 cause type IV mucolipidosis, a devastating pediatric neurodegenerative disease. Our recent electrophysiological studies revealed that, although a TRPML1-mediated current can only be recorded in late endosome and lysosome (LEL) using the lysosome patch clamp technique, a proline substitution in TRPML1 (TRPML1V432P) results in a large whole cell current. Thus, it remains unknown whether the large TRPML1V432P-mediated current results from an increased surface expression (trafficking), elevated channel activity (gating), or both. Here we performed systemic Pro substitutions in a region previously implicated in the gating of various 6 transmembrane cation channels. We found that several Pro substitutions displayed gain-of-function (GOF) constitutive activities at both the plasma membrane (PM) and endolysosomal membranes. Although wild-type TRPML1 and non-GOF Pro substitutions localized exclusively in LEL and were barely detectable in the PM, the GOF mutations with high constitutive activities were not restricted to LEL compartments, and most significantly, exhibited significant surface expression. Because lysosomal exocytosis is Ca2+-dependent, constitutive Ca2+ permeability due to Pro substitutions may have resulted in stimulus-independent intralysosomal Ca2+ release, hence the surface expression and whole cell current of TRPML1. Indeed, surface staining of lysosome-associated membrane protein-1 (Lamp-1) was dramatically increased in cells expressing GOF TRPML1 channels. We conclude that TRPML1 is an inwardly rectifying, proton-impermeable, Ca2+ and Fe2+/Mn2+ dually permeable cation channel that may be gated by unidentified cellular mechanisms through a conformational change in the cytoplasmic face of the transmembrane 5 (TM5). Furthermore, activation of TRPML1 in LEL may lead to the appearance of TRPML1 proteins at the PM.
Plasticity of user interfaces: towards an evolution model based on conceptual graphs

This paper deals with Plasticity of User Interfaces (UI). Plasticity refers to the ability of UIs to withstand variations of context of use while preserving usability. By context of use, we mean a <user, platform, environment< triplet. Adaptation is a three-step process involving recognition of the situation, computation of a reaction to cope with the situation, and execution of the reaction. This paper covers both computation and execution of the reaction. It proposes a graph of descriptions and an evolution model for respectively capitalizing UIs and specifying the adaptation to perform in case of change of context of use. These notions are based on conceptual graphs.
kin-19/casein kinase Iα has dual functions in regulating asymmetric division and terminal differentiation in C. elegans epidermal stem cells

Casein Kinase I (CKI) is a conserved component of the Wnt signaling pathway, which regulates cell fate determination in metazoans. We show that post-embryonic asymmetric division and fate specification of C. elegans epidermal stem cells are controlled by a non-canonical Wnt/b-catenin signaling pathway, involving the b-catenins WRM-1 and SYS-1, and that C. elegans kin-19/CKIa functions in this pathway. Furthermore, we find that kin-19 is the only member of the Wnt asymmetry pathway that functions with, or in parallel to, the heterochronic temporal patterning pathway to control withdrawal from self-renewal and subsequent terminal differentiation of epidermal stem cells. We show that, except in the case of kin-19, the Wnt asymmetry pathway and the heterochronic pathway function separately and in parallel to control different aspects of epidermal stem cell fate specification. However, given the function of kin-19/CKIa in both pathways, and that CKI, Wnt signaling pathway and heterochronic pathway genes are widely conserved in animals, our findings suggest that CKIa may function as a regulatory hub through which asymmetric division and terminal differentiation are coordinated in adult stem cells of vertebrates.