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Abstract The first case of CWD in a Norwegian red deer was detected by a routine ELISA test and confirmed by western blotting and immunohistochemistry in the brain stem of the animal. Two different western blotting tests were conducted independently in two different laboratories, showing that the red deer glycoprofile was different from the Norwegian CWD reindeer and CWD moose and from North American CWD. The isolate showed nevertheless features similar to the classical BSE (BSE-C) strain. Furthermore, BSE-C could not be excluded based on the PrPSc immunohistochemistry staining in the brainstem and the absence of detectable PrPSc in the lymphoid tissues. Because of the known ability of BSE-C to cross species barriers as well as its zoonotic potential, the CWD red deer isolate was submitted to the EURL Strain Typing Expert Group (STEG) as a BSE-C suspect for further investigation. In addition, different strain typing in vivo and in vitro strategies aiming at identifying the BSE-C strain in the red deer isolate were performed independently in three research groups and BSE-C was not found in it. These results suggest that the Norwegian CWD red deer case was infected with a previously unknown CWD type and further investigation is needed to determine the characteristics of this potential new CWD strain.

Dado el entorno empresarial actual, altamente competitivo, las empresas necesitan empleados comprometidos, que tengan comportamientos innovadores y que contribuyan a la innovación de la organización. En este sentido, proponemos el constructo Liderazgo para la Innovación, un estilo específico de liderazgo que promueve comportamientos innovadores en los empleados. Asimismo, presentamos el proceso de construcción y validación de la Escala de Liderazgo para la Innovación (ILS-16, por su sigla en inglés), a través de tres estudios secuenciales, analizando sus propiedades psicométricas. Los estudios se realizaron con diferentes muestras, para un total de 367 empleados. Los resultados demostraron que la ILS-16 es una escala unidimensional válida y confiable para evaluar el constructo propuesto. Este estudio tiene implicaciones para los académicos que tendrán la posibilidad de construir nuevos modelos y para los gerentes, en tanto que la ILS-16 constituye una herramienta práctica para medir el liderazgo para la innovación en sus organizaciones.

Abstract Background More than 40 pathogenic heterozygous PRNP mutations causing inherited prion diseases have been identified to date. Recessive inherited prion disease has not been described to date. Methods We describe the clinical and neuropathological data of inherited early-onset prion disease caused by the rare PRNP homozygous mutation R136S. In vitro PrPSc propagation studies were performed using recombinant-adapted protein misfolding cyclic amplification technique. Brain material from two R136S homozygous patients was intracranially inoculated in TgMet129 and TgVal129 transgenic mice to assess the transmissibility of this rare inherited form of prion disease. Results The index case presented symptoms of early-onset dementia beginning at the age of 49 and died at the age of 53. Neuropathological evaluation of the proband revealed abundant multicentric PrP plaques and Western blotting revealed a ~ 8 kDa protease-resistant, unglycosylated PrPSc fragment, consistent with a Gerstmann-Sträussler-Scheinker phenotype. Her youngest sibling suffered from progressive cognitive decline, motor impairment, and myoclonus with onset in her late 30s and died at the age of 48. Genetic analysis revealed the presence of the R136S mutation in homozygosis in the two affected subjects linked to homozygous methionine at codon 129. One sibling carrying the heterozygous R136S mutation, linked to homozygous methionine at codon 129, is still asymptomatic at the age of 74. The inoculation of human brain homogenates from our index case and an independent case from a Portuguese family with the same mutation in transgenic mice expressing human PrP and in vitro propagation of PrPSc studies failed to show disease transmissibility. Conclusion In conclusion, biallelic R136S substitution is a rare variant that produces inherited early-onset human prion disease with a Gerstmann-Sträussler-Scheinker neuropathological and molecular signature. Even if the R136S variant is predicted to be “probably damaging”, heterozygous carriers are protected, at least from an early onset providing evidence for a potentially recessive pattern of inheritance in human prion diseases.

Abstract Pigs are susceptible to infection with the classical bovine spongiform encephalopathy (C-BSE) agent following experimental inoculation, and PrPSc accumulation was detected in porcine tissues after the inoculation of certain scrapie and chronic wasting disease isolates. However, a robust transmission barrier has been described in this species and, although they were exposed to C-BSE agent in many European countries, no cases of natural transmissible spongiform encephalopathies (TSE) infections have been reported in pigs. Transmission of atypical scrapie to bovinized mice resulted in the emergence of C-BSE prions. Here, we conducted a study to determine if pigs are susceptible to atypical scrapie. To this end, 12, 8–9-month-old minipigs were intracerebrally inoculated with two atypical scrapie sources. Animals were euthanized between 22- and 72-months post inoculation without clinical signs of TSE. All pigs tested negative for PrPSc accumulation by enzyme immunoassay, immunohistochemistry, western blotting and bioassay in porcine PrP mice. Surprisingly, in vitro protein misfolding cyclic amplification demonstrated the presence of C-BSE prions in different brain areas from seven pigs inoculated with both atypical scrapie isolates. Our results suggest that pigs exposed to atypical scrapie prions could become a reservoir for C-BSE and corroborate that C-BSE prions emerge during interspecies passage of atypical scrapie.

Abstract E/D163 polymorphism of dog prion protein (PrP) has been recently proposed as the variant responsible for canid prion resistance. To further investigate the protective role of this variant against prion replication, the transgenic mouse model OvPrP-Tg532 expressing sheep/goat PrP carrying the substitution D162 (equivalent to D163 position of dog PrP) was generated and intracranially inoculated with a broad collection of small ruminant prion strains. OvPrP-Tg532 mice showed resistance to classical bovine spongiform encephalopathy (BSE) from sheep and some classical scrapie isolates from sheep and goat but were susceptible to ovine atypical L-BSE and numerous classical scrapie isolates. Strikingly, some of these classical scrapie isolates showed a shift in their prion strain properties. These results suggest that other PrP residues apart from E/D163 variant of dog PrP or factors distinct than PrP may participate in prion resistance of canids and that different factors may be required for D162 sheep PrP to provide effective protection to sheep against ruminant prions.

Abstract The diversity of goat scrapie strains in Europe has recently been studied using bioassays in a wide collection of rodent models, resulting in the classification of classical scrapie into four different categories. However, the sole use of the first passage does not lead to isolate adaptation and identification of the strains involved and might therefore lead to misclassification of some scrapie isolates. Therefore, this work reports the complete transmission study of a wide collection of goat transmissible spongiform encephalopathy (TSE) isolates by intracranial inoculation in two transgenic mouse lines overexpressing either small ruminant (TgGoat-ARQ) or bovine (TgBov) PrPC. To compare scrapie strains in sheep and goats, sheep scrapie isolates from different European countries were also included in the study. Once the species barrier phenomenon was overcome, an accurate classification of the isolates was attained. Thus, the use of just two rodent models allowed us to fully differentiate at least four different classical scrapie strains in small ruminants and to identify isolates containing mixtures of strains. This work reinforces the idea that classical scrapie in small ruminants is a prion disease caused by multiple different prion strains and not by a single strain, as is the case for epidemic classical bovine spongiform encephalopathy (BSE-C). In addition, the clear dissimilarity between the different scrapie strains and BSE-C does not support the idea that classical scrapie is the origin of epidemic BSE-C.

Abstract Current classifications of sporadic Creutzfeldt–Jakob disease (sCJD) identify five subtypes associated with different disease phenotypes. Most of these histopathological phenotypes (histotypes) co-distribute with distinct pairings of methionine (M)/valine (V) genotypes at codon 129 of the prion protein (PrP) gene and the type (1 or 2) of the disease-associated PrP (PrPD). Types 1 and 2 are defined by the molecular mass (~ 21 kDa and ~ 19 kDa, respectively) of the unglycosylated isoform of the proteinase K-resistant PrPD (resPrPD). We recently reported that the sCJDVV1 subtype (129VV homozygosity paired with PrPD type 1, T1) shows an electrophoretic profile where the resPrPD unglycosylated isoform is characterized by either one of two single bands of ~ 20 kDa (T120) and ~ 21 kDa (T121), or a doublet of ~ 21–20 kDa (T121−20). We also showed that T120 and T121 in sCJDVV have different conformational features but are associated with indistinguishable histotypes. The presence of three distinct molecular profiles of T1 is unique and raises the issue as to whether T120 and T121 represent distinct prion strains. To answer this question, brain homogenates from sCJDVV cases harboring each of the three resPrPD profiles, were inoculated to transgenic (Tg) mice expressing the human PrP-129M or PrP-129V genotypes. We found that T120 and T121 were faithfully replicated in Tg129V mice. Electrophoretic profile and incubation period of mice challenged with T121−20 resembled those of mice inoculated with T121 and T120, respectively. As in sCJDVV1, Tg129V mice challenged with T121 and T120 generated virtually undistinguishable histotypes. In Tg129M mice, T121 was not replicated while T120 and T121−20 generated a ~ 21–20 kDa doublet after lengthier incubation periods. On second passage, Tg129M mice incubation periods and regional PrP accumulation significantly differed in T120 and T121−20 challenged mice. Combined, these data indicate that T121 and T120 resPrPD represent distinct human prion strains associated with partially overlapping histotypes.

Classical bovine spongiform encephalopathy (BSE) is the only zoonotic prion disease described to date. Although the zoonotic potential of atypical BSE prions have been partially studied, an extensive analysis is still needed. We conducted a systematic study by inoculating atypical BSE isolates from different countries in Europe into transgenic mice overexpressing human prion protein (PrP): TgMet129, TgMet/Val129, and TgVal129. L-type BSE showed a higher zoonotic potential in TgMet129 mice than classical BSE, whereas Val129-PrP variant was a strong molecular protector against L-type BSE prions, even in heterozygosis. H-type BSE could not be transmitted to any of the mice. We also adapted 1 H- and 1 L-type BSE isolate to sheep-PrP transgenic mice and inoculated them into human-PrP transgenic mice. Atypical BSE prions showed a modification in their zoonotic ability after adaptation to sheep-PrP producing agents able to infect TgMet129 and TgVal129, bearing features that make them indistinguishable of sporadic Creutzfeldt-Jakob disease prions.

Múltiples modelos de madurez en tecnologías de información (ti) son presentados en la literatura. Algunos han sido validados en pymes y pocos operacionalizan el método para clasificar las organizaciones en niveles de madurez. La mayoría de estos hacen una clasificación general de las organizaciones en un único nivel de madurez. Sin embargo, una organización podría estar en múltiples niveles de madurez a la vez para diferentes criterios de clasificación. Utilizando la capacidad en ti para analizar la madurez tecnológica desde diferentes dimensiones, se aplica un algoritmo de agrupación jerárquico (hcpc) para agrupar 2.470 empresas estudiadas. A partir de esta agrupación, se identificaron cuatro niveles de madurez para cada una de las dimensiones de la capacidad en ti: nivel 1 o de incorporación, nivel 2 o de aprendizaje, nivel 3 o de planeación y control, y nivel 4 o de alineación. Finalmente, se propone un método para clasificar las organizaciones en estos cuatro niveles de madurez para cada una de las dimensiones de la capacidad en ti.

Neurodegenerative diseases (NDs) are some of the most important health challenges modern medicine and advanced societies face. Indeed, the number of patients affected by one of these illnesses will increase in the following years at the same rate that human life expectancy allows us to live longer. Despite many years of research, NDs remain invariably fatal. A complete understanding of the exact mechanisms leading to neuronal death, which will ideally allow preclinical detection and the development of effective treatments, has not yet been achieved. However, a great deal of information about ND pathology and the search for possible therapies has been acquired using animal models and more precisely transgenic mouse models. In this review, the main contributions of these powerful research tools in NDs as well as their advantages and caveats are discussed.

La capacidad en tecnologías de información (TI) se reconoce como un conjunto de recursos en TI y su sinergia, que podrán potenciar a las organizaciones en el desarrollo de sus capacidades organizacionales y en la consecución de ventajas competitivas sostenibles. Múltiples definiciones de este constructo han sido propuestas; sin embargo, las condiciones particulares de las micros, pequeñas y medianas empresas (mipymes) hacen necesario proponer y validar un mode­lo que permita evaluar la capacidad en TI en este tipo de contextos. A partir de un análisis factorial exploratorio y confirmatorio, sobre una muestra de 517 mipymes, se valida un modelo de capacidad en TI. El modelo resultante describe la capacidad en TI en cuatro categorías principales: capacidad de cohesión organizacional del área de TI (categoría I), capacidad estratégica en TI (categoría II), infraestructura de TI (categoría III) y capacidad de relacionamiento externo (categoría IV), siendo las categorías I y II las que tienen una influencia más significativa sobre la capacidad de TI. La categoría I está representada por el conocimiento organizacional del personal de TI y su capacidad de relacionamiento al interior de la organización. La categoría II está descrita por el conocimiento estratégico de las TI y su capacidad de planeación estratégica en TI. Este modelo es una base para la medición de la capacidad en ti de las mipymes, así como para la generación de políticas públicas.

Prion propagation can be interfered with by the expression of a second prion protein in the host. In the present study, we investigated prion propagation in a host expressing two different prion protein genes.

Background: Fatal Familial Insomnia (FFI) is a genetic prion disease caused by the D178N mutation in the prion protein gene (PRNP) in coupling phase with methionine at PRNP 129. In 2017, we have shown that the olfactory mucosa (OM) collected from FFI patients contained traces of PrPSc detectable by Protein Misfolding Cyclic Amplification (PMCA). Methods: In this work, we have challenged PMCA-generated products obtained from OM and brain homogenate of FFI patients in BvPrP-Tg407 transgenic mice expressing the bank vole prion protein to test their ability to induce prion pathology. Results: All inoculated mice developed mild spongiform changes, astroglial activation, and PrPSc deposition mainly affecting the thalamus. However, their neuropathological alterations were different from those found in the brain of BvPrP-Tg407 mice injected with raw FFI brain homogenate. Conclusions: Although with some experimental constraints, we show that PrPSc present in OM of FFI patients is potentially infectious. Funding: This work was supported in part by the Italian Ministry of Health (GR-2013-02355724 and Ricerca Corrente), MJFF, ALZ, Alzheimer’s Research UK and the Weston Brain Institute (BAND2015), and Euronanomed III (SPEEDY) to FM; by the Spanish Ministerio de Economía y Competitividad (grant AGL2016-78054-R [AEI/FEDER, UE]) to JMT and JCE; AM-M was supported by a fellowship from the INIA (FPI-SGIT-2015-02).

Resumen Se indaga sobre las prácticas sociales que diferentes actores del municipio de Ambalema realizan en el centro histórico, declarado Bien de Interés Cultural (BIC), con el propósito de reconocer cómo se apropian del patrimonio urbano arquitectónico, para la generación de propuestas que contribuyan, no solo a conservar y salvaguardar los bienes patrimoniales, sino al mejoramiento de las condiciones de vida de la comunidad. A través de encuestas, grupos focales y entrevistas a profundidad se preguntó a los habitantes y diferentes miembros del lugar sobre dichas prácticas, a fin de entender, a nivel social, el acelerado deterioro que sufren varios de los inmuebles representativos del municipio. La investigación concluyó que las labores conservacionistas realizadas por los diferentes actores son de un nivel básico, tales como mantenimiento general y arreglos locativos; se encontró una tendencia general de pasividad frente a las acciones de preservación del bien de interés cultural.Palabras clave: apropiación social, bien de interés cultural, conservación, centro histórico, patrimonio arquitectónico y urbano.Social practices in cultural heritage of Ambalema, TolimaAbstractThis article investigates the social practices of different actors in the historic city center of the municipality of Ambalema, declared as an Asset of Cultural Interest, aiming to recognize how they make use of the urban architectural heritage in order to formulate proposals that would contribute not only to the conservation and safeguarding of patrimonial assets, but also to the improvement of living conditions in the community. Through surveys, focus groups, and in-depth interviews, the inhabitants and different members of the local community were asked about their practices, seeking to understand the social significance of the accelerated deterioration of some of the representative properties in the municipality. The research concluded that conservation work carried out by different actors rarely goes beyond basic efforts, such as general maintenance and local repairs, evidencing a general tendency to passivity regarding the preservation of this asset of cultural interest.Keywords: Asset of cultural interest, social appropriation, conservation, historical center, architectural and urban heritage.Práticas sociais ante o patrimônio cultural em Ambalema, TolimaResumo Indaga-se sobre as práticas sociais que diferentes atores do município de Ambalema (Tolima, Colômbia) realizam no centro histórico, declarado Bem de Interesse Cultural, com o propósito de reconhecer como se apropriam do patrimônio urbano arquitetônico, para a geração de propostas que contribuam não somente para conservar e proteger os bens patrimoniais, mas também para melhorar as condições de vida da comunidade. Por meio de enquetes, grupos focais e entrevistas a profundidade, perguntou-se aos moradores e aos diferentes membros do lugar sobre essas práticas a fim de entender, no contexto social, a acelerada deterioração que vários dos imóveis representativos do município sofrem. A pesquisa concluiu que os trabalhos de conservação realizados pelos diferentes atores são de um nível básico, como manutenção geral e consertos pontuais; constatou-se uma tendência geral de passividade diante das ações de preservação do bem de interesse cultural.Palavras-chave: apropriação social, bem de interesse cultural, centro histórico, conservação, patrimônio arquitetônico e urbano.Recibido: diciembre 14 / 2016 Evaluado: septiembre 25 / 2017 Aceptado: octubre 17 / 2017

Bovine spongiform encephalopathy (BSE) is the only known zoonotic prion that causes variant Creutzfeldt-Jakob disease (vCJD) in humans. The major risk determinant for this disease is the polymorphic codon 129 of the human prion protein (Hu-PrP), where either methionine (Met129) or valine (Val129) can be encoded. To date, all clinical and neuropathologically confirmed vCJD cases have been Met129 homozygous, with the exception of 1 recently reported Met/Val heterozygous case. Here, we found that transgenic mice homozygous for Val129 Hu-PrP show severely restricted propagation of the BSE prion strain, but this constraint can be partially overcome by adaptation of the BSE agent to the Met129 Hu-PrP. In addition, the transmission of vCJD to transgenic mice homozygous for Val129 Hu-PrP resulted in a prion with distinct strain features. These observations may indicate increased risk for vCJD secondary transmission in Val129 Hu-PrP–positive humans with the emergence of new strain features.

The aim of this study was to determine if entrepreneurial intention, based on Ajzen’s model of planned behavior (1991), can be predicted by risk propensity, internal locus of control and leadership skills. Six standardized and reliable instruments were applied to 1493 undergraduate university business students in Latin American countries, selected through non-random quota sampling in accordance with their formation level in each of the five participating universities. Using structural equation techniques, the research model was validated and intention estimated and analyzed in relation to a set of socio-demographic variables. According to the results, entrepreneurial intention can be significantly predicted by the psychological variables under consideration and, contrary to what has been reported in other research, no gender differences were found in the intention of entrepreneurship. These findings are discussed.

The aim of this study was to examine the validity of the Spiritual Leadership model proposed by Fry (2003). Spiritual Leadership Questionnaire [SLT] (Fry & Matherly, 2006) was applied to 279 workers from Colombia and Ecuador. The two samples were equivalent by sex, age, education level and work experience. According to the results, all dimensions of the questionnaire showed to be highly consistent (alpha above .85) except Commitment dimension. Likewise, it found that just one of 35 items that composed the scale shows low item-scale correlation. In the most of the cases, the relationships between variables as well as the adjustment index of the model were lower than reported by Fry previously, which limited the construct validity. External evidences of validity of the model, demonstrated that the work experience and level of charge weresignificant for Spiritual Leadership dimensions. These findings were related to the psychometrics requirement of the scale andregarding of measured construct with organizational behavior. Finally, it is point out some research limitations and provide some suggestions for future studies.

Energy crop expansion can increase land demand and generate displacement of food crops, which impacts greenhouse gas (GHG) emissions mainly through land-use change (LUC). Increased agricultural productivity could compensate for this. Our study aims to evaluate the regional combined GHG emissions of increasing agricultural yields for food crop and beef production and using the generated surplus land for biomass production to replace fossil fuels in the Orinoquia region of Colombia until 2030. The results show that surplus land for biomass production is obtained only when strong measures are applied to increase agricultural productivity. In the medium and high scenario, a land surplus of 0.6 and 2.4 Mha, respectively, could be generated. Such intensification results in up to 83% emission reduction in Orinoquia’s agricultural sector, largely coming from increasing productivity of cattle production and improving degraded pastures. Biofuel potential from the surplus land is projected at 36 to 368 PJ per year, with a low risk of causing indirect LUC, and results in GHG emission reductions of more than 100% compared to its fossil fuel equivalent. An integrated perspective of the agricultural land use enables sustainable production of both food and bioenergy.

El objetivo de este estudio es observar si existe un patrón característico de liderazgo en una cultura regional específica y analizar si se presentan diferencias significativas en razón del sector económico al que pertenecen las organizaciones (industria y servicio). Se trabajó con una muestra de 449 empleados de ocho empresas que operan en la ciudad de Bucaramanga. Se utilizó el Test de adjetivos de Pitcher —PAT—. De acuerdo con los resultados, sí existe un patrón de liderazgo caracterizado por los adjetivos: responsable, humano, trabajador, colaborador, honesto y amable. En general, las características de liderazgo en los dos sectores estudiados fueron muy similares, aunque se observaron algunas diferencias. En este texto se discuten estos hallazgos.

El propósito de este estudio fue analizar, a través del MLQ 5X, los perfiles de liderazgo de 167 directivos de cuatro países latinoamericanos (Perú, Venezuela, Ecuador y Colombia), para observar si a pesar de la similitud cultural se presentan diferencias en los patrones de liderazgo. Se compararon estos perfiles con los obtenidos por otros autores en regiones culturalmente distintas, para finalmente confrontar los resultados con los de otra muestra colombiana. Se evidenciaron diferencias significativas en liderazgo, tanto entre países culturalmente distintos, como entre los mismos países latinoamericanos, a pesar de su similitud cultural. Finalmente, la muestra de directivos de este estudio difiere significativamente de la otra muestra colombiana, respecto a algunas variables del MLQ. Se discuten esos hallazgos.

Variably protease-sensitive prionopathy (VPSPr), a recently identified and seemingly sporadic human prion disease, is distinct from Creutzfeldt-Jakob disease (CJD) but shares features of Gerstmann-Sträussler-Scheinker disease (GSS). However, contrary to exclusively inherited GSS, no prion protein (PrP) gene variations have been detected in VPSPr, suggesting that VPSPr might be the long-sought sporadic form of GSS. The VPSPr atypical features raised the issue of transmissibility, a prototypical property of prion diseases. We inoculated VPSPr brain homogenate into transgenic mice expressing various levels of human PrP (PrPC). On first passage, 54% of challenged mice showed histopathologic lesions, and 34% harbored abnormal PrP similar to that of VPSPr. Surprisingly, no prion disease was detected on second passage. We concluded that VPSPr is transmissible; thus, it is an authentic prion disease. However, we speculate that normal human PrPC is not an efficient conversion substrate (or mouse brain not a favorable environment) and therefore cannot sustain replication beyond the first passage.

El propósito de este estudio fue determinar si la intención de emprendimiento, con base en el modelo de comportamiento planeado de Ajzen (1991), puede predecirse con base en la propensión al riesgo, el locus de control interno y las habilidades de liderazgo. Asimismo, se identificaron variables sociodemográficas asociadas a tal intención. Se aplicó una batería de instrumentos estandarizados y confiables a 1493 estudiantes universitarios de áreas empresariales en cinco países latinoamericanos. De acuerdo con los resultados, la intención de emprendimiento puede predecirse significativamente con las variables estudiadas. De igual forma, se confirmó la incidencia de los antecedentes familiares en tal intención lo cual ha sido reportado en varios estudios. Contrario a lo hallado en otros estudios, no se encontraron diferencias por género en la intención de emprendimiento. Se discuten estos hallazgos.

Los continuos e impredecibles cambios que deben afrontar las organizaciones en la actualidad les exigen asumir retos cada vez más complejos que les garanticen resultados para sobrevivir en un mercado altamente competitivo. Tal condición requiere de directivos que optimicen sus habilidades para liderar equipos, considerando unas características que antes parecían irrelevantes. El propósito de éste estudio es delimitar conceptualmente el liderazgo en relación con los equipos de alto desempeño y discutir entorno al rol que este debe tener en su conformación y mantenimiento. Se puntualiza sobre la necesidad de que el líder se convierta en un facilitador para la transformación no solo de las personas que componen los equipos, sino dela organización, a través de desarrollo y crecimiento. Se plantea la necesidad de incluir en los procesos de dirección exitosa de equipos de alto desempeño factores como la estrategia organizacional, la gestión humana y la estructura organizacional, todos ellos permeados necesariamente por los estilos de liderazgo y la actitud del líder hacia el cambio.

La presente investigación buscó conocer las diferentes estrategias de afrontamiento empleadas por las familias frente a las secuelas generadas en los pacientes que sufrieron Trauma Craneoencefálico (TCE) en el segundo semestre de 2010; para esto se realizó un estudio cualitativo con enfoque fenomenológico, aplicado a una muestra de 15 cuidadores principales de pacientes que sufrieron Trauma Craneoencefálico (TCE). El método utilizado para la recolección de la información fue la entrevista a profundidad, manejando como instrumento de ayuda la grabadora de voz y las notas de campo. El análisis utilizado fue de contenido tomando como referencia las etapas de afrontamiento familiar desorganización, recuperación, reorganización. La familia es el agente principal de la recuperación prolongada de la persona lesionada y las adaptaciones a la discapacidad. La relación de consanguinidad encontrada en la mayor parte de los cuidadores fue (madre - hijo). Las estrategias más utilizadas por los cuidadores fueron: aceptación de la responsabilidad, independencia y vida personal, tensión emocional familiar, autocontrol y reevaluación positiva.

Se describen 3 casos de pacientes con linfoma MALT, diagnosticados, tratados y seguidos en la consulta de hematología del Hospital Provincial Docente Clinicoquirúrgico "Saturnino Lora" de Santiago de Cuba, a la cual fueron remitidos por gastroenterólogos, otorrinolaringólogos y maxilofaciales de la mencionada institución. Uno de los afectados presentaba una masa tumoral gástrica y en nasofaringe, aparecida en diferentes momentos; otro un tumor linfoide en el paladar duro, que recurrió en ganglios infradiafragmáticos; y un tercero un nódulo linfoide en la glándula salival parótida unilateral, con recidiva en ganglios regionales después de haber sido extirpado. Todos experimentaron una buena respuesta clínica al inicio del tratamiento convencional, pero en 2 de ellos se confirmaron reapariciones no locales del proceso morboso.Three cases of patients with MALT lymphoma are described, who were diagnosed, treated and followed up at the hematology department of "Saturnino Lora" Teaching Provincial Hospital in Santiago de Cuba, to which they were referred by gastroenterologists, otolaryngologists and maxillofacial specialists of that institution. One of those patients presented with a nasopharyngeal and gastric mass, which appeared at different times; another patient had lymphoid tumor of the hard palate, which recurred in infradiaphragmatic lymph nodes; and a third one had a lymphoid node in the unilateral salivary parotid gland with recurrence in regional nodes after having been removed. All experienced a good clinical response at the beginning of conventional treatment, but in 2 of them non-local recurrences of the disease process were confirmed.

El presente artículo da cuenta de un proceso de revisión documental basado en estudios y algunas teorías relacionadas con el concepto de aptitud física de corredores aęcionados y su relación con la salud. En este sentido, recopila, sistematiza, compara y establece bases teóricas, documentales y conceptuales que favorezcan la discusión sobre los hallazgos y conclusiones de estudios dirigidos al tema. En este orden de ideas, el interés fundamental del texto se dirige a la identięcación del concepto de aptitud física en corredores aęcionados y los peręles ęsiológicos que reflejan la salud de estos individuos, así como la descripción de los factores relacionados con la práctica deportiva, que pueden llegar a incidir en las condiciones de bienestar de estos deportistas. Asimismo, se incluye en esta revisión reportes de investigación empírica y teórica publicada en diferentes revistas del área de la medicina deportiva y del entrenamiento deportivo.

Las Redes Neuronales Artificiales se aplican en la estimación o predicción de los sistemas de detección de fallas de equipos, logrando con ellas las mismas funciones que el modelo estadístico tradicional. Se argumentó un método para sustituir los algoritmos de selección y el conjunto de modelos que normalmente se utilizan en sistemas de control predictivo de procesos complejos, los que están determinados por un conjunto de variables, con un amplio rango de variación de cada una de ellas, como es el caso del extractable del níquel obtenido en los hornos de reducción de la fábrica, por una Red Neuronal.

El propósito de este estudio fue describir la personalidad, el afecto y la inteligencia emocional a lo largo de la formación profesional, en 422 estudiantes de áreas empresariales. Se utilizó el Inventario de Personalidad NEO-FFI de Costa & McCrae (1994), el TMMS-24 (adaptado por Fernández-Berrocal, Extremera & Ramos, 2004) y el PANAS (adaptado por Sandín et al., 1999). Se encontraron niveles altos de neuroticismo y bajos de extraversión, apertura a la experiencia y amabilidad; niveles intermedios de inteligencia emocional y una afectividad positiva predominante. El neuroticismo se incrementó en algunos semestres, la apertura a la experiencia y la responsabilidad aunque presentan niveles bajos, tienden a incrementarse durante la formación. Los participantes se caracterizaron por su afectividad positiva, la cual no parece depender de los procesos formativos sino de sus características personales. Se discuten estos hallazgos en relación con la formación de líderes empresariales.

Este estudio tuvo como propósito determinar si las variables psicológicas percepción de autoeficacia y ansiedad guardan relación con el rendimiento académico en un grupo de 120 estudiantes de secundaria de un colegio privado de Bogotá. Para ello, se aplicó la Escala de Autoeficacia Generalizada [EAG] y el Cuestionario de Ansiedad Estado - Rasgo [STAI]. Los resultados evidenciaron que la autoeficacia está asociada directamente con el rendimiento académico general, mientras que la ansiedad no. Al examinar por áreas de conocimiento, se encontró que tanto la autoeficacia como la ansiedad resultan ser significativas para la predicción del rendimiento académico. Se discute el papel contextual de la ansiedad así como de su posible mediación en la autoeficacia y el rendimiento académico.

Se estudió la creatividad en 115 niños mediante la Prueba de Creatividad desarrollada por De Zubiría, Marlés y Ramírez (2003), observando la incidencia del género y de la edad sobre los resultados. Adicionalmente, se estimó la confiabilidad y la validez de la prueba. Los resultados indicaron que el género no presenta un efecto significativo en los puntajes de la prueba, mientras que la edad resultó ser un factor importante en los procesos creativos de los niños. Se hacen recomendaciones respecto a la especificidad de la edad en los baremos de esta prueba.

The population of wine yeasts during spontaneous must fermentation was characterized by direct 5.8S-ITS rDNA region amplification without previous plate isolation or enrichment. RFLP analysis was applied to each of the amplification products detected, and the corresponding yeast identifications were made. The method provides a fast and direct way of determining yeast population present during wine fermentation.

A new variant of Creutzfeldt Jacob Disease (vCJD) was identified in humans and linked to the consumption of Bovine Spongiform Encephalopathy (BSE)-infected meat products. Recycling of ruminant tissue in meat and bone meal (MBM) has been proposed as origin of the BSE epidemic. During this epidemic, sheep and goats have been exposed to BSE-contaminated MBM. It is well known that sheep can be experimentally infected with BSE and two field BSE-like cases have been reported in goats. In this work we evaluated the human susceptibility to small ruminants-passaged BSE prions by inoculating two different transgenic mouse lines expressing the methionine (Met) allele of human PrP at codon 129 (tg650 and tg340) with several sheep and goat BSE isolates and compared their transmission characteristics with those of cattle BSE. While the molecular and neuropathological transmission features were undistinguishable and similar to those obtained after transmission of vCJD in both transgenic mouse lines, sheep and goat BSE isolates showed higher transmission efficiency on serial passaging compared to cattle BSE. We found that this higher transmission efficiency was strongly influenced by the ovine PrP sequence, rather than by other host species-specific factors. Although extrapolation of results from prion transmission studies by using transgenic mice has to be done very carefully, especially when human susceptibility to prions is analyzed, our results clearly indicate that Met129 homozygous individuals might be susceptible to a sheep or goat BSE agent at a higher degree than to cattle BSE, and that these agents might transmit with molecular and neuropathological properties indistinguishable from those of vCJD. Our results suggest that the possibility of a small ruminant BSE prion as vCJD causal agent could not be ruled out, and that the risk for humans of a potential goat and/or sheep BSE agent should not be underestimated.

In the past, natural scrapie and bovine spongiform encephalopathy (BSE) infections have essentially not been diagnosed in sheep homozygous for the A136R154R171 haplotype of the prion protein. This genotype was therefore assumed to confer resistance to BSE and classic scrapie under natural exposure conditions. Hence, to exclude prions from the human food chain, massive breeding efforts have been undertaken in the European Union to amplify this gene. We report the identification of 2 natural scrapie cases in ARR/ARR sheep that have biochemical and transmission characteristics similar to cases of classic scrapie, although the abnormally folded prion protein (PrPSc) was associated with a lower proteinase-K resistance. PrPSc was clearly distinct from BSE prions passaged in sheep and from atypical scrapie prions. These findings strongly support the idea that scrapie prions are a mosaic of agents, which harbor different biologic properties, rather than a unique entity.

ABSTRACT Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently classified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K-digested abnormal prion protein (PrPres) isoform identified by Western blotting (type 1 or type 2). Converging evidence led to the view that MM/MV1, VV/MV2, and VV1 and MM2 sCJD cases are caused by distinct prion strains. However, in a significant proportion of sCJD patients, both type 1 and type 2 PrPres were reported to accumulate in the brain, which raised questions about the diversity of sCJD prion strains and the coexistence of two prion strains in the same patient. In this study, a panel of sCJD brain isolates (n = 29) that displayed either a single or mixed type 1/type 2 PrPres were transmitted into human-PrP-expressing mice (tgHu). These bioassays demonstrated that two distinct prion strains (M1CJD and V2CJD) were associated with the development of sCJD in MM1/MV1 and VV2/MV2 patients. However, in about 35% of the investigated VV and MV cases, transmission results were consistent with the presence of both M1CJD and V2CJD strains, including in patients who displayed a “pure” type 1 or type 2 PrPres. The use of a highly sensitive prion in vitro amplification technique that specifically probes the V2CJD strain revealed the presence of the V2CJD prion in more than 80% of the investigated isolates, including isolates that propagated as a pure M1CJD strain in tgHu. These results demonstrate that at least two sCJD prion strains can be present in a single patient. IMPORTANCE sCJD occurrence is currently assumed to result from spontaneous and stochastic formation of a misfolded PrP nucleus in the brains of affected patients. This original nucleus then recruits and converts nascent PrPC into PrPSc, leading to the propagation of prions in the patient’s brain. Our study demonstrates the coexistence of two prion strains in the brains of a majority of the 23 sCJD patients investigated. The relative proportion of these sCJD strains varied both between patients and between brain areas in a single patient. These findings strongly support the view that the replication of an sCJD prion strain in the brain of a patient can result in the propagation of different prion strain subpopulations. Beyond its conceptual importance for our understanding of prion strain properties and evolution, the sCJD strain mixture phenomenon and its frequency among patients have important implications for the development of therapeutic strategies for prion diseases.

MicroRNAs (miRNAs) may contribute to the development and pathology of many neurodegenerative diseases, including prion diseases. They are also promising biomarker candidates due to their stability in body fluids. We investigated miRNA alterations in a Tg501 mouse model of prion diseases that expresses a transgene encoding the goat prion protein (PRNP). Tg501 mice intracranially inoculated with mouse-adapted goat scrapie were compared with age-matched, mock inoculated controls in preclinical and clinical stages. Small RNA sequencing from the cervical spinal cord indicated that miR-223-3p, miR-151-3p, and miR-144-5p were dysregulated in scrapie-inoculated animals before the onset of symptoms. In clinical-stage animals, 23 significant miRNA alterations were found. These miRNAs were predicted to modify the Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways including prion disease, extracellular matrix interactions, glutaminergic synapse, axon guidance, and transforming growth factor-beta signaling. MicroRNAs miR-146a-5p (up in cervical spinal cord) and miR-342-3p (down in cervical spinal cord, cerebellum and plasma), both indicated in neurodegenerative diseases earlier, were verified by quantitative real-time polymerase chain reaction (qRT-PCR). Minimal changes observed before the disease onset suggests that most miRNA alterations observed here are driven by advanced prion-associated pathology, possibly limiting their use as diagnostic markers. However, the results encourage further mechanistic studies on miRNA-regulated pathways involved in these neurodegenerative conditions.

Using different prion strains, such as the variant Creutzfeldt-Jakob disease agent and the atypical bovine spongiform encephalopathy agents, and using transgenic mice expressing human or bovine prion protein, we assessed the reliability of protein misfolding cyclic amplification (PMCA) to model interspecies and genetic barriers to prion transmission. We compared our PMCA results with in vivo transmission data characterized by attack rates, i.e., the percentage of inoculated mice that developed the disease. Using 19 seed/substrate combinations, we observed that a significant PMCA amplification was only obtained when the mouse line used as substrate is susceptible to the corresponding strain. Our results suggest that PMCA provides a useful tool to study genetic barriers to transmission and to study the zoonotic potential of emerging prion strains.

We generated transgenic mice expressing bovine cellular prion protein (PrPC) with a leucine substitution at codon 113 (113L). This protein is homologous to human protein with mutation 102L, and its genetic link with Gerstmann–Sträussler–Scheinker syndrome has been established. This mutation in bovine PrPC causes a fully penetrant, lethal, spongiform encephalopathy. This genetic disease was transmitted by intracerebral inoculation of brain homogenate from ill mice expressing mutant bovine PrP to mice expressing wild-type bovine PrP, which indicated de novo generation of infectious prions. Our findings demonstrate that a single amino acid change in the PrPC sequence can induce spontaneous generation of an infectious prion disease that differs from all others identified in hosts expressing the same PrPC sequence. These observations support the view that a variety of infectious prion strains might spontaneously emerge in hosts displaying random genetic PrPC mutations.

Bovine spongiform encephalopathy (BSE) and BSE-related disorders have been associated with a single major prion strain. Recently, 2 atypical, presumably sporadic forms of BSE have been associated with 2 distinct prion strains that are characterized mainly by distinct Western blot profiles of abnormal protease-resistant prion protein (PrPres), named high-type (BSE-H) and low-type (BSE-L), that also differed from classical BSE. We characterized 5 atypical BSE-H isolates by analyzing their molecular and neuropathologic properties during transmission in transgenic mice expressing homologous bovine prion protein. Unexpectedly, in several inoculated animals, strain features emerged that were highly similar to those of classical BSE agent. These findings demonstrate the capability of an atypical bovine prion to acquire classical BSE–like properties during propagation in a homologous bovine prion protein context and support the view that the epidemic BSE agent could have originated from such a cattle prion.

How susceptible pigs are to infection with sheep prions is unknown. We show, through transmission experiments in transgenic mice expressing porcine prion protein (PrP), that the susceptibility of this mouse model to bovine spongiform encephalopathy (BSE) can be enhanced after its passage in ARQ sheep, indicating that the pathogenicity of the BSE agent is modified after passage in sheep. Transgenic mice expressing porcine PrP were, nevertheless, completely resistant to infection with a broad panel of classical scrapie isolates from different sheep PrP genotypes and with different biochemical characteristics. The atypical (Nor98 like) isolate (SC-PS152) was the only scrapie isolate capable of transmission in these mice, although with a marked transmission barrier. Unexpectedly, the atypical scrapie agent appeared to undergo a strain phenotype shift upon transmission to porcine-PrP transgenic mice and acquired new strain properties, suggesting that atypical scrapie agent may exhibit different phenotypes depending on the host cellular PrP or other genetic factors.

The specific characteristics of Transmissible Spongiform Encephalopathy (TSE) strains may be altered during passage across a species barrier. In this study we investigated the biochemical and biological characteristics of Bovine Spongiform Encephalopathy (BSE) after transmission in both natural host species (cattle, sheep, pigs and mice) and in transgenic mice overexpressing the corresponding cellular prion protein (PrPC) in comparison with other non-BSE related prions from the same species. After these passages, most features of the BSE agent remained unchanged. BSE-derived agents only showed slight modifications in the biochemical properties of the accumulated PrPSc, which were demonstrated to be reversible upon re-inoculation into transgenic mice expressing bovine-PrPC. Transmission experiments in transgenic mice expressing bovine, porcine or human-PrP revealed that all BSE-derived agents were transmitted with no or a weak transmission barrier. In contrast, a high species barrier was observed for the non-BSE related prions that harboured an identical PrP amino acid sequence, supporting the theory that the prion transmission barrier is modulated by strain properties (presumably conformation-dependent) rather than by PrP amino acid sequence differences between host and donor. As identical results were observed with prions propagated either in natural hosts or in transgenic mouse models, we postulate that the species barrier and its passage consequences are uniquely governed by the host PrPC sequence and not influenced by other host genetic factors. The results presented herein reinforce the idea that the BSE agent is highly promiscuous, infecting other species, maintaining its properties in the new species, and even increasing its capabilities to jump to other species including humans. These data are essential for the development of an accurate risk assessment for BSE.