Aproximación a la obra poética de Ramón Xirau: Entre filosofía y mística

A lo largo de la segunda mitad del siglo XX Ramón Xirau, en su actividad como filósofo, maestro, crítico literario, editor, escritor y pensador, se ha confirmado como un autor clave para la comprensión de la Historia del Pensamiento Iberoamericano y de la Historia de la Literatura Latinoamericana, en particular, mexicana. Este trabajo quiere ser una aproximación a su obra poética, gracias a la cual hoy en día es conocido como uno de los principales poetas en lengua catalana. Se analizarán especialmente aquellos elementos de su poética que, conceptualizados, podemos encontrar también en sus obras filosófica y que relacionan de modo esencial poesía, filosofía y místic

De Catalunya al mundo. Trayectoria intelectual de José Ferrater Mora

Tesis doctoral inédita leída en la Universidad Autónoma de Madrid, Facultad de Filosofía y Letras, Departamento de Antropología Social y Pensamiento Filosófico Español. Fecha de lectura: 02-12-2016Esta tesis tiene embargado el acceso al texto completo hasta el 02-06-2018En este trabajo hemos llevado a cabo un análisis diacrónico e interdisciplinar de la entera trayectoria intelectual de José Ferrater Mora, de acuerdo con la hipótesis metodológica según la cual la obra de un autor tiene que ser estudiada en su totalidad y teniendo en cuenta el contexto que ha permitido su desarrollo. En modo particular, hemos orientado nuestro estudio hacia dos direcciones inéditas: por un lado, hacia el descubrimiento de la actividad de Ferrater durante su “etapa catalana”, eso es, antes de la guerra civil; por el otro, hacia el análisis de su producción artística en el ámbito de la poesía, la narrativa, la cinematografía y la fotografía. Nuestro análisis se ha basado sobre cuatro tipos de fuentes: la bibliografía secundaria sobre el autor, la bibliografía del mismo Ferrater, las fuentes documentales de archivo y el testimonio directo de las personas que tuvieron contacto con él. El primer tipo de fuente, que ha sido indispensable para acercarnos a la obra de Ferrater, ha sido objeto de un largo estudio crítico, donde hemos puesto de manifiesto los tópicos que han protagonizado los “estudios ferraterianos”, así como los vacíos bibliográficos producidos por esos mismos tópicos. La producción de Ferrater ha sido sometida a un estudio de corte hermenéutico e histórico-filosófico, que nos ha servido para reconstruir el desarrollo de las principales temáticas de interés filosófico e intelectual que han animado su trayectoria intelectual. La investigación en archivos nos ha permitido reconstruir por primera vez de forma detallada la biografía y la circunstancia de Ferrater, desde los años catalanes hasta su periodo estadounidense. Asimismo, nos ha permitido encontrar varios documentos inéditos, reproducidos integralmente en los anexos documentales de la tesis. Finalmente, las entrevistas con algunos de los mejores especialistas en su obra, así como con personas de su entorno íntimo, nos han permitido averiguar detalles biográficos y contextuales que han arrojado ulterior luz sobre la interpretación de su obra. A través de esta investigación, hemos así podido revelar muchos elementos inéditos de la trayectoria intelectual de José Ferrater Mora, a la luz de los cuales hemos podido ofrecer una nueva interpretación de su entera actividad intelectual.In questo lavoro abbiamo condotto un’analisi diacronica e interdisciplinare dell’intera traiettoria intellettuale di José Ferrater Mora, seguendo l’ipotesi metodologica secondo la quale l’opera di un’autore dev’essere studiata nella sua interezza, tenendo conto del contesto che ha permesso il suo sviluppo. In modo particolare, abbiamo orientato il nostro studio in due direzioni inedite: da una parte, verso la scoperta dell’attività di Ferrater Mora durante la sua “tappa catalana”, cioè, durante gli anni anteriori alla guerra civile; dall’altra parte,verso l’analisi della sua produzione artistica, nell’ambito della poesia, della narrativa, della cinematografia e della fotografia. La nostra analisi si è basata su quattro tipi di fonti: la bibliografia secondaria intorno all’autore, la bibliografia dello stesso Ferrater, le fonti documentali d’archivio e la testimonianza diretta delle persone che hanno vissuto a contatto con lui. Il primo tipo di fonte, che è risultato indispensabile per avvicinarci all’opera di Ferrater, è stato oggetto di un amplio studio, in cui abbiamo mostrato i topici che hanno protagonizzato gli “studi ferrateriani”, così come i vuoti bibliografici prodotti da questi stessi topici. La produzione di Ferrater è stata sottomessa a uno studio di tipo ermeneutico e storico-filosofico, che ci è servito per ricostruire lo sviluppo delle principali tematiche d’interesse filosofico e intellettuale che hanno caratterizzato la sua traiettoria intellettuale. La ricerca d’archivio ci ha permesso di ricomporre per la prima volta in modo dettagliato la biografia e la circostanza di Ferrater, dagli anni catalani sino al suo periodo statunitense. Allo stesso modo, ci ha permesso di scoprire vari documenti inediti, riprodotti integralmente negli allegati documentali della tesi. Per ultimo, le interviste con alcuni dei migliori specialisti nel suo pensiero, così come con alcune delle persone del suo ambito privato, ci hanno permesso di recuperare dettagli biografici e contestuali che hanno gettato ulteriore luce sull’interpretazione della sua opera. Attraverso questa ricerca, abbiamo quindi potuto mostrare molti elementi inediti della traiettoria intellettuale di José Ferrater Mora, alla luce dei quali abbiamo potuto offrire una nuova interpretazione della sua intera attività intellettuale

Dinâmica do uso das terras no Cerrado no período de 2002 a 2013

The objective of this work was to analyze land use dynamics in the Brazilian Cerrado region from 2002 to 2013. This analysis was based on the interpretation of Landsat satellite images carried out by the projects Projeto de Conservação e Utilização Sustentável da Diversidade Biológica Brasileira (Probio) and TerraClass Cerrado 2013, both coordinated by Ministério do Meio Ambiente. In 2002, 38.9% of the Cerrado was covered by some type of anthropic activity. In 2013, this percentage increased to 43.4%. One of the main highlights is the emergence of a new agricultural frontier in the northern region of the study area, known as Matopiba.O objetivo deste trabalho foi analisar a dinâmica de uso das terras na região do Cerrado de 2002 a 2013. Essa análise foi baseada nas interpretações de imagens do satélite Landsat realizadas pelo Projeto de Conservação e Utilização Sustentável da Diversidade Biológica Brasileira (Probio) e pelo projeto TerraClass Cerrado 2013, coordenados pelo Ministério do Meio Ambiente. Em 2002, 38,9% do Cerrado eram cobertos por algum tipo de atividade antrópica. Em 2013, essa percentagem passou para 43,4%. Um dos grandes destaques é o surgimento de uma nova fronteira agrícola na região norte da área de estudo, conhecida como Matopiba

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p

Filosofía de la historia e historia de la filosofía en la obra de Ramón Xirau

L’opera di Ramon Xirau, autore appartenente alla seconda generazione d’esiliati spagnoli del 1939, ha influito profondamente nella storia del pensiero in lingua spagnola della seconda meta del XX secolo, soprattutto, nel contesto iberoamericano e messicano. Sono stati particolarmente influenti i suoi lavori di carattere storico-filosofico, che hanno accompagnato nello studio della filosofia oltre due generazioni di autori latinoamericani. Con questo lavoro vogliamo porre in risalto gli elementi caratteristici della metodologia che Xirau applica nelle sue opere storico-filosofiche, sottolineando la sua relazione con un’originale concezione di una filosofia della storia.La obra de Ramón Xirau, autor perteneciente a la segunda generación de exiliados españoles de 1939, ha marcado profundamente la historia del pensamiento en lengua española de la segunda mitad del siglo XX, sobre todo en el contexto iberoamericano y mexicano. Particularmente influyentes han sido sus estudios de carácter histórico-filosófico, que han acompañado a más de dos generaciones de autores latinoamericanos en el estudio de la filosofía. Este trabajo se propone poner de relieve los elementos mas característicos del enfoque metodológico adoptado por Ramón Xirau en sus trabajos historico-filosóficos, haciendo hincapié sobre la relación que mantienen con su original concepción de la filosofía de la historia

Sulle tracce di un catalano universale

The main goal of this paper is to reconstruct José Ferrater Mora’s intellectual trajectory. Ferrater Mora is an important Catalan exiled thinker and he has a big influence in the contemporary Spanish society and in the history of universal philosophy. This article wants to demonstrate the importance of analysing not only his philosophical books and essays but also his literary and cinematographic works. At the same time, it wants to emphasize how Ferrater Mora’s life and works are proofs of the possibility of coexistence between local and universal culture in the same author

The unfolded protein response in amyotrophic later sclerosis: results of a phase 2 trial

: Strong evidence suggests that endoplasmic reticulum stress plays a critical role in the pathogenesis of amyotrophic lateral sclerosis (ALS) through altered regulation of proteostasis. Robust preclinical findings demonstrated that guanabenz selectively inhibits endoplasmic reticulum stress-induced eIF2\u3b1-phosphatase, allowing misfolded protein clearance, reduces neuronal death and prolongs survival in in vitro and in vivo models. However, its safety and efficacy in patients with ALS are unknown. To address these issues, we conducted a multicentre, randomized, double-blind trial with a futility design. Patients with ALS who had displayed an onset of symptoms within the previous 18\u2009months were randomly assigned in a 1:1:1:1 ratio to receive 64\u2009mg, 32\u2009mg or 16\u2009mg of guanabenz or placebo daily for 6\u2009months as an add-on therapy to riluzole. The purpose of the placebo group blinding was to determine safety but not efficacy. The primary outcome was the proportion of patients progressing to higher stages of disease within 6\u2009months as measured using the ALS Milano-Torino staging system, compared with a historical cohort of 200 patients with ALS. The secondary outcomes were the rate of decline in the total revised ALS functional rating scale score, slow vital capacity change, time to death, tracheotomy or permanent ventilation and serum light neurofilament level at 6\u2009months. The primary assessment of efficacy was performed using intention-to-treat analysis. The treatment arms using 64\u2009mg and 32\u2009mg guanabenz, both alone and combined, reached the primary hypothesis of non-futility, with the proportions of patients who progressed to higher stages of disease at 6\u2009months being significantly lower than that expected under the hypothesis of non-futility and a significantly lower difference in the median rate of change in the total revised ALS functional rating scale score. This effect was driven by patients with bulbar onset, none of whom (0/18) progressed to a higher stage of disease at 6\u2009months compared with those on 16\u2009mg guanabenz (4/8; 50%), the historical cohort alone (21/49; 43%; P\u2009=\u20090.001) or plus placebo (25/60; 42%; P\u2009=\u20090.001). The proportion of patients who experienced at least one adverse event was higher in any guanabenz arm than in the placebo arm, with higher dosing arms having a significantly higher proportion of drug-related side effects and the 64\u2009mg arm a significantly higher drop-out rate. The number of serious adverse events did not significantly differ between the guanabenz arms and the placebo. Our findings indicate that a larger trial with a molecule targeting the unfolded protein response pathway without the alpha-2 adrenergic related side-effect profile of guanabenz is warranted

Association of Variants in the SPTLC1 Gene with Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation. Objective: To identify the genetic variants associated with juvenile ALS. Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism. Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members. Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway. Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.

Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356

non present