Las miopatías metabólicas son un grupo de trastornos genéticos que disminuyen la capacidad del músculo esquelético para utilizar sustratos energéticos y sintetizar ATP. Estas alteraciones pueden clasificarse en tres tipos fundamentalmente: i) trastornos del metabolismo de los carbohidratos (del glucógeno y de la glucosa), ii) defectos del metabolismo lipídico, y iii) alteraciones de la fosforilación oxidativa –OXPHOS-. Las dos primeras se deben a deficiencias enzimáticas de las rutas metabólicas de degradación y síntesis de glúcidos y lípidos y muestran diversas manifestaciones (...) clínicas, pero una buena parte de ellas cursan con intolerancia al ejercicio. Aunque un buen número de pacientes con estos trastornos musculares presentan síntomas en la infancia, el diagnóstico normalmente se retrasa hasta la segunda y tercera década de la vida. Por tanto, reconocer las características clínicas de estas deficiencias conduce a un diagnóstico precoz y a un mejor tratamiento. Las enfermedades mitocondriales son un grupo de trastornos originados por una deficiencia en la síntesis de ATP a través del sistema de fosforilación oxidativa. Este sistema está formado por proteínas codificadas en los dos genomas de la célula (nuclear y mitocondrial) y, por tanto, pueden presentar un modelo de herencia mendeliano o materno. En esta revisión se describirán las características especiales del sistema genético mitocondrial y las principales mutaciones que causan enfermedades en humanos. (shrink)

Esta entrevista se realizó a través de Zoom el 13 de julio de 2021.

The present article is framed within the biosemiotic glossary project as a way to address common terminology within biosemiotic research. The glossary integrates the view of the members of the biosemiotic community through a standard survey and a literature review. The concept of ‘semiotic threshold’ was first introduced by Umberto Eco, defining it as a boundary between semiotic and non-semiotic areas. We review here the concept of ‘semiotic threshold’, first describing its denotation within semiotics via an examination on the history (...) of the concept, its synonyms, antonyms, etymology, usage in other languages and context in which it is used. Then we present a general overview of the survey among researchers, analyzing the difference in responses for the concept of ‘lower semiotic threshold’ and related concepts. From the answers we also review the difference between the general usage of ‘semiotic threshold’ versus its specific use within biosemiotics, and attempt to make a general synthesis of the concept taking into account what we have learned from the survey and the literature review. (shrink)

The prevalent idea that semiosis is evolutionary is a driving point for biosemiotic research, starting from the Peircean premises of continuity and including a large number of views on how signs evolve. In this paper I wish to add a small pinch of skepticism to an otherwise productive point of view. Briefly, the question to be asked is: Is there any proper and fair connection between the logical abstraction of signs, genetic expressions interpreted as signs and the animal usage of (...) signs? And how do we go about answering this? Instead of attempting a negative account of the possibility of an evolutionary view of biosemiosis, I will attempt to make an argument in favor of skepticism as a way to make a more fine-grained distinction across the areas where biosemiotic thinking seems to have some impact. The aim is then to find philosophical strategies to overcome this skepticism when possible, while also raising some awareness about the possible limits of current biosemiotics regarding the ideal evolutionary chain of signs. Ultimately, the idea is reexamining some core assumptions of the biosemiotic point of view at its most general, accounting for some possible ways in which theory may move forward. The potential incompatibility of theoretical standpoints between some of the different approaches that may be taken is, it will be argued, a desirable outcome for biosemiotic research. That is, the way we deal with the possible theories on the evolutionary continuity of signs will also affect our different research programs, and having a nuanced philosophical discussion on it can only contribute to the expansion and clarification of where different positions within biosemiotics currently stand. (shrink)

Resumen En este artículo se examina el Darwinismo Neural en su explicación de la evolución de la conciencia humana, contrastando su metodología con la utilizada por Richard Lewontin, Richard Levins y Steven Rose, quienes han hecho importantes aportaciones en el estudio de los sistemas vivos desde un punto de vista dialéctico. Concluimos que la explicación interaccionista de la evolución de la conciencia planteada en el DN, supera muchas de las deficiencias del determinismo biológico; sin embargo, al compartir algunos lineamientos con (...) el cartesianismo, mantiene un carácter a-histórico insuficiente para la explicación de la conciencia humana. Finalmente, proponemos la adopción de una teoría explicativa de la conciencia humana que considere las relaciones existentes en ella, no sólo como interacciones sino también como interpenetraciones ontogénicas y filogenéticas.This article examines Neural Darwinism proposed as an explanation of evolution of human consciousness. His methodology is contrasted with that used by Richard Lewontin, Richard Levins, and Steven Rose, who made important contributions to the study of living systems from a dialectical point of view. We conclude that the interactionist explanation of the evolution of conscience put forward in ND overcomes many of the deficiencies proper to biological determinism. However, as ND also shares many of the foundations of the Cartesian approach, it maintains a non-historical character, insufficient for the account of human consciousness. We suggest the adoption of an alternative theory, able to fully explain human conscience which must consider the whole of existing relations in it, and, instead of merely considering them as interactions, they have to be observed as dialectical interpenetrations. (shrink)

Resumen: Este artículo pretende abordar aspectos esenciales del pensamiento de Henri Bergson en la dimensión de las prácticas políticas. Específicamente, esta indagación se centrará en un análisis del sentido de la democracia en Les Deux Sources de la morale et de la religion, en las escuetas, pero decisivas porciones de texto donde esta es abordada. Bajo este objetivo, se podrá extender una discusión de fondo, de carácter ontológico, en torno al rendimiento de una noción de Acontecimiento que pretende servir de (...) reflexión basal en torno al dominio de un concepto de lo político atribuible a este filósofo francés.: This article aims to address essential aspects of Henri Bergson’s thought in the dimension of political practices. Specifically, this inquiry will focus on an analysis of the meaning of democracy in Les Deux Sources de la morale et de la religion, in the succinct, but decisive portions of the text where it is focussed. Under this point, it will be possible to extend a substantive discussion, of an ontological nature, around the performance of a notion of Event that aims to serve as a basal reflection on the domain of a concept of the political attributable to this French philosopher. (shrink)

The Annual Biosemiotic Achievement Award was established at the annual meeting of the International Society for Biosemiotic Studies in 2014, in conjunction with Springer and Biosemiotics. It seeks to recognize papers published in the journal that present novel and potentially important contributions to biosemiotic research, its scientific impact and its future prospects. Here the winner of the Biosemiotic Achievement Award for 2020 is announced: The award goes to Filip Jaroš and Matěj Pudil for the article “Cognitive systems of human and (...) non-human animals: At the crossroads of phenomenology, ethology and biosemiotics”. (shrink)

Cognitive Science, Volume 46, Issue 5, May 2022.

We examined whether auditors' attitude to the evidence may be driven by their perception of the self-fulfilling prophecy effect. Following previous research on motivated reasoning, we assumed that the self-fulfilling prophecy effect could be interpreted as a potential motivational/incentive factor supporting auditor's reluctance to release going concern opinions. We contribute to the literature by demonstrating in a laboratory experiment that auditors' perceptions of the self-fulfilling prophecy effect can bias their professional judgment. To this extend, the Hogarth and Einhorn's belief-adjustment model (...) was employed in order to estimate auditors' attitudes in regards to both aggravating and mitigating evidence. Our results indicate that the fear of provoking the self-fulfilling prophecy creates a directional goal which leads auditors to process the audit evidence in a manner more likely to yield the desired conclusion. Auditors' motivated by the fear of causing the self-fulfilling prophecy effect offered a greater sensitivity to the positive evidence and, at the same time, a lower tendency to favor negative evidence. We also provide a discussion of the implications of our results for both regulators and the auditing profession. (shrink)

ABSTRACT In this article we analyze the attempt that young Simone Weil made for thinking time and change, and for deciphering the participation that the world and the mind may have in its configuration. To achieve this, we set out to elucidate two concepts that we believe synthesize her approach: the first one is astonishment, understood both in its quality of incessant intuition that reveals the human impotence to understand the present, and in its quality as a mediating instance between (...) ignorance and approximate human science of the phenomenal field. The second concept we analyze is that of directed time, significant of the first Weilian philosophy in that it reveals the subjugation of man to the present, the only tangible time. This determination allows us to understand the meaning of the past as an abolished existence, and the future as a virtual nucleus of possibilities towards which the human being prepares his action and work thanks to his faculty to devise projects. Finally, we try to show the problematic tension that young Weil discerned between the law of the immediacy of the world, and the law of time that imposes intermediate steps to the realization of all human activity. (shrink)

This project reflects on the way in which students in a situation of social risk construct their identity. Based on the reflections and theories originating from research conducted on individuals and collective groups in a situation of social exclusion due to disability, social class or ethnicity, this paper will analyse the conflicts these students have to deal with when constructing their identity. It also examines the challenge that education has to face to turn those conflicts into opportunities that will help (...) to build life projects with which they can freely identify. For this reason, from a critical perspective, the school’s role in constructing identity will be analysed, as will the way in which it affects children and adolescents from minority groups. In the same way, we will study and put forward some different channels aimed at providing more equal educational attention to those identities that are depreciated in neoliberal society. (shrink)

La creatividad y la paz están íntimamente relacionadas, incluso en su dimensión histórica. El cambio de concepción de la paz observado por Galtung lleva a una comparación con la evolución de la noción de creatividad. Además de las mencionadas similitudes, ambos conceptos están en concomitancia en tanto en cuanto la creatividad es necesaria como herramienta para la transformación de conflictos de forma pacífica. El nexo que se propone para unir ambos conceptos es la educación, encardinada dentro de un constructo que (...) llamamos triángulo abstractivo. (shrink)

Relativistic geometrical action for a quantum particle in the superspace is analyzed from theoretical group point of view. To this end an alternative technique of quantization outlined by the authors in a previous work and that is based in the correct interpretation of the square root Hamiltonian, is used. The obtained spectrum of physical states and the Fock construction consist of Squeezed States which correspond to the representations with the lowest weights $\lambda=\frac{1}{4}$ and $\lambda=\frac{3}{4}$ with four possible (non-trivial) fractional representations (...) for the group decomposition of the spin structure. From the theory of semigroups the analytical representation of the radical operator in the superspace is constructed, the conserved currents are computed and a new relativistic wave equation is proposed and explicitly solved for the time-dependent case. The relation with the Relativistic Schrödinger equation and the Time-dependent Harmonic Oscillator is analyzed and discussed. (shrink)

La inteligencia artificial (IA) no es sólo una moda, sino un conjunto de sistemastecnológicos que han adquirido en los últimos años un enorme desarrollo con aplicaciones a múltiples aspectos de la vida cotidiana de los ciudadanos, tanto por parte de particulares (empresas) como de los propios Estados.La Unión Europea ha apostado siempre por compatibilizar los avances tecnológicos con el respeto de los principios y valores de la democracia, el Estado de Derecho y los derechos y libertades fundamentales. Desde tales premisas, (...) actualmente se enfrenta a la búsqueda de un modelo regulatorio de la IA ante las carencias del Reglamento General de Protección de Datos (RGPD) en este ámbito.A través de la Propuesta de Reglamento (UE) sobre IA, se pretende adoptar una regulación marco del uso de la IA que suponga una “tercera vía” inspirada en los principios y valores de la Unión y se contraponga a las actuales fórmulas regulatorias “abstencionistas” (EE.UU) e “hiperreguladoras” (China), proporcionando un modelo que consiga el efecto imitador o “efecto Bruselas” de una IA “confiable” para los ciudadanos y las empresas a nivel mundial. (shrink)

While naturalism is used in positive senses by the tradition of analytical philosophy, with Ludwig Wittgenstein its best example, and by the tradition of phenomenology, with Maurice Merleau-Ponty its best exemplar, it also has an extremely negative sense on both of these fronts. Hence, both Merleau-Ponty and Wittgenstein in their basic thrusts adamantly reject reductionistic naturalism. Although Merleau-Ponty’s phenomenology rejects the naturalism Husserl rejects, he early on found a place for the “truth of naturalism.” In a parallel way, Wittgenstein accepts (...) a certain positive sense of naturalism, while rejecting Quine’s kind of naturalism. It is the aim of this paper to investigate the common ground in the views of Wittgenstein and Merleau-Ponty regarding the naturalism that they each espouse and that which they each reject. (shrink)

A finalidade do texto seguinte consiste na exposição de uma relação de promiscuidade entre a técnica e a ciência na obra de Jean-François Lyotard. A primeira etapa deste artigo consiste na apresentação de um problema referente à obra A condição pós-moderna, onde a noção de técnica aparece sobre uma perspectiva positivista. Aqui, a intimidade entre técnica e ciência é resultado da crise dos fundamentos das ciências na modernidade, crise oriunda do afastamento entre as ciências e os discursos que se prestavam (...) frequentemente à sua legitimação. Esses discursos, que Lyotard aproxima do modelo narrativo, e dos quais se destacam o discurso político de emancipação e o discurso filosófico especulativo, entram em declínio na condição pós-moderna. As ciências, desprovidas de motivações ou destinos humanistas, não resistem à lógica capitalista, e encontram o intermediário de sua relação com o capital na técnica. O critério da ciência deixa, pouco a pouco, de ser a busca pela verdade e passa a ser a busca pelo melhor desempenho. A segunda e conclusiva etapa do artigo, consiste, por sua vez, na radicalização dessa promiscuidade, e se refere principalmente ao livro O inumano. A assim chamada tecnociência corresponde à completa submissão da ciência à técnica: se a visão positivista de A condição pós-moderna vê na técnica um suplemento, uma prótese que dá assistência à pesquisa científica, no segundo livro, a ciência se mostra a prótese ou o suplemento da técnica, que se revela, não obstante, inumana. Nessa segunda parte do artigo, pretendo mostrar que a visão positivista da técnica não é, portanto, suficiente para compreender a relação promíscua que ela tem com a ciência, mostrando-se necessário o recurso à perspectiva clássica da mesma: é a partir da visão da técnica como produtividade cega da physis que alcançamos também o entendimento do caráter inumano da tecnociência. (shrink)

The prediction of legal judgments is one of the most recognized fields in Natural Language Processing, Artificial Intelligence, and Law combined. By legal prediction, we mean intelligent systems capable of predicting specific judicial characteristics such as the judicial outcome, the judicial class, and the prediction of a particular case. In this study, we used an artificial intelligence classifier to predict the decisions of Brazilian courts. To this end, we developed a text crawler to extract data from official Brazilian electronic legal (...) systems, consisting of two datasets of cases of second-degree murder and active corruption. We applied various classifiers, such as Support Vector Machines, Neural Networks, and others, to predict judicial outcomes by analyzing text features from the dataset. Our research demonstrated that Regression Trees, Gated Recurring Units, and Hierarchical Attention Networks tended to have higher metrics across our datasets. As the final goal, we searched the weights of one of the algorithms, Hierarchical Attention Networks, to find samples of the words that might be used to acquit or convict defendants based on their relevance to the algorithm. (shrink)

O presente artigo trata do caráter contingente ou necessário da relação causal entre a causa primeira e seu efeito, tal como apresentada por Avicena e Tomás de Aquino. Para tanto, aborda o paralelismo no tratamento da inteligência e vontade divinas por parte de ambos os autores. Ambos aceitam que Deus conhece a si mesmo e, conhecendo-se, conhece aquilo que lhe é distinto. Aceitam igualmente que Deus quer ou ama a si mesmo e, querendo a si mesmo, quer acidentalmente o que (...) lhe é distinto. No entanto, discordam com respeito à necessidade ou contingência do mundo. Para Avicena, o mundo é possível em si mesmo, mas “necessário por outro”. Para Tomás, que Deus queira o mundo com necessidade hipotética corresponde mais à necessidade do querer divino, sem que isso determine a contingência ou necessidade do mundo. Com efeito, na visão tomista, é preferível um universo no qual haja mescla de necessidade e contingência, pois isso representa melhor as múltiplas perfeições divinas. (shrink)

En medio de la potencial relación entre Fernando González y la Filosofía se presenta un recorrido por su obra escrita, y desde ella, sus hermenéuticas, viajes, retratos, métodos, parábolas, vivencias y contemplaciones. Ello con el fin de culminar en una exhortación metodológica para los nuevos estudios sobre su obra desde perspectivas particulares, para no hallarse como muchos en la paradoja de lo parcial y de la anécdota en medio de una obra armónica; si bien este ensayo es una reflexión a (...) partir de otra posible lectura parcial. (shrink)

Segundo o autor do artigo, a teoria do juízo reflexivo que se encontra na primeira parte da Crítica do Juízo de Kant dedicada ao gosto estético representa uma busca do sentido do dado na experiência. Este sentido não pode ser posto, antes tem de ser descoberto, pois a experiência do belo, a criação estética da arte, não podem ser compreendidos mediante preconceitos que já possuímos. Este tipo de juízo é o que se requer também segundo Arendt diante dos acontecimentos e (...) situações sempre novas da vida política e da história. As condições da comunicabilidade universal do juízo reflexivo são os apelos ao sentido comum humano e a amplitude de pensamento necessária para assumir o ponto de vista dos outros. O artigo mostra também serem estas as condições que, segundo Arendt, se requerem para uma autêntica compreensão política. (shrink)

En el presente trabajo se reflexiona sobre la situación de pobreza y desigualdad que asola al mundo en este nuevo siglo señalando el papel que desarrolla la Universidad en el cambio social y en la consecución de los Objetivos de Desarrollo Sostenible. Las Universidades se enfrentan a un gran desafío, contribuir a la creación de un mundo sostenible e igualitario. La Educación Superior se convierte en guía para una sociedad que necesita, hoy más que nunca, de una conciencia crítica que (...) les haga actuar desde la responsabilidad socy el compromiso ético. En esta investigación, se expone un caso concreto, la asignatura de Educación para Responsabilidad Social impartida en la Universidad Francisco de Vitoria como una materia que ayuda a los estudiantes a implicarse personalmente para lograr la justicia social y el bien común, trabajando activamente para incidir en el logro de los ODS. (shrink)