Apuntes jurídicos sobre el paisaje lingüístico en Euskal Herria

This paper is a study of place-names and signs in the Basque Country from the point of view of language law. These are matters that relate to both the status and corpus of language and contribute to the formation of the language landscape,» After a brief historical introduction, the author focuses on the factors that bear on signs and the language 1andscape: the cornpetence factor and the language factor. The description of the latter leads the author to a discussion of the existing language system, in which the Spanish and Basque sharing official status does not necessarily entail the obligation to use both languages at the same time. Using this discussion as a frame of reference, the au- thor analyses place-names, traffic signals and signs. As for place-names, the existing rules are deemed rigid and lacking in ambition, in that they do not pursue the dissemination of official Basque forms. In traffic signaIs, Basque has to appear alongside Spanish, which is required by Spanish legislation, although this bilingualism excludes place-names that have an official Basque form only. With regard to signs, the author analyses public premises, companies licensed to provide public services and the private sector. For public premises there is no specific regulation, but the status of Basque as an autochthonous language, together with the identification and informatíon purposes of signs, could support the exclusive use of this language, According to the author , companies licensed to provide public services should observe the same language system as the goverment and therefore promote the use of Basque. Finally, in the private sector, the author upholds the legitimacy of measures to promote Basque language use such as tax allowances and exemptions in advertising and commercial signs.

Las ferrerías guipuzcoanas ante la crisis

[ES]Durante mucho tiempo la historiografía ha considerado que la actividad siderometalúrgica vasca y guipuzcoana sufrió una manifiesta crisis durante el siglo XVII. Sin embargo, un análisis profundo de la documentación, más allá de los meros testimonios e indicios, demuestra que el XVII fue un período dinámico para la industria del hierro, en el que se produjeron innovaciones, transformaciones y reajustes continuos que permitieron al sector seguir manteniendo su prestigio en los mercados mundiales.

Income Taxation and Growth in an OLG Economy: Does Aggregate Uncertainty Play any Role?

We analyze the effects of capital income taxation on long-run growth in a stochastic, two-period overlapping generations economy. Endogenous growth is driven by a positive externality of physical capital in the production sector that makes firms exhibit an aggregate technology in equilibrium. We distinguish between capital income and labor income, and between attitudes towards risk and intertemporal substitution of consumption. We show necessary and sufficient conditions such that i) increments in the capital income taxation lead to higher equilibrium growth rates, and ii) the effect of changes in the capital income tax rate on the equilibrium growth may be of opposite signs in stochastic and in deterministic economies. Such a sign reversal is shown to be more likely depending on i) how the intertemporal elasticity of substitution compares to one, and ii) the size of second- period labor supply. Numerical simulations show that for reasonable values of the intertemporal elasticity of substitution, a sign reversal shows up only for implausibly high values of the second- period’s labor supply. The conclusion is that deterministic OLG economies are a good approximation of the effect of taxes on the equilibrium growth rate as in Smith (1996).

Contribution of Pannexin1 to Experimental Autoimmune Encephalomyelitis

Pannexin1 (Panx1) is a plasma membrane channel permeable to relatively large molecules, such as ATP. In the central nervous system (CNS) Panx1 is found in neurons and glia and in the immune system in macrophages and T-cells. We tested the hypothesis that Panx1-mediated ATP release contributes to expression of Experimental Autoimmune Encephalomyelitis (EAE), an animal model for multiple sclerosis, using wild-type (WT) and Panx1 knockout (KO) mice. Panx1 KO mice displayed a delayed onset of clinical signs of EAE and decreased mortality compared to WT mice, but developed as severe symptoms as the surviving WT mice. Spinal cord inflammatory lesions were also reduced in Panx1 KO EAE mice during acute disease. Additionally, pharmacologic inhibition of Panx1 channels with mefloquine (MFQ) reduced severity of acute and chronic EAE when administered before or after onset of clinical signs. ATP release and YoPro uptake were significantly increased in WT mice with EAE as compared to WT non-EAE and reduced in tissues of EAE Panx1 KO mice. Interestingly, we found that the P2X7 receptor was upregulated in the chronic phase of EAE in both WT and Panx1 KO spinal cords. Such increase in receptor expression is likely to counterbalance the decrease in ATP release recorded from Panx1 KO mice and thus contribute to the development of EAE symptoms in these mice. The present study shows that a Panx1 dependent mechanism (ATP release and/or inflammasome activation) contributes to disease progression, and that inhibition of Panx1 using pharmacology or gene disruption delays and attenuates clinical signs of EAE.

In Vitro and In Vivo Evaluation of a Folate-Targeted Copolymeric Submicrohydrogel Based on N-Isopropylacrylamide as 5-Fluorouracil Delivery System

Folate-targeted poly[(p-nitrophenyl acrylate)-co-(N-isopropylacrylamide)] nanohydrogel (F-SubMG) was loaded with 5-fluorouracil (5-FU) to obtain low (16.3 +/- 1.9 mu g 5-FU/mg F-SubMG) and high (46.8 +/- 3.8 mu g 5-FU/mg F-SubMG) load 5-FU-loaded F-SubMGs. The complete in vitro drug release took place in 8 h. The cytotoxicity of unloaded F-SubMGs in MCF7 and HeLa cells was low; although it increased for high F-SubMG concentration. The administration of 10 mu M 5-FU by 5-FU-loaded F-SubMGs was effective on both cellular types. Cell uptake of F-SubMGs took place in both cell types, but it was higher in HeLa cells because they are folate receptor positive. After subcutaneous administration (28 mg 5-FU/kg b.w.) in Wistar rats, F-SubMGs were detected at the site of injection under the skin. Histological studies indicated that the F-SubMGs were surrounded by connective tissue, without any signs of rejections, even 60 days after injection. Pharmacokinetic study showed an increase in MRT (mean residence time) of 5-FU when the drug was administered by drug-loaded F-SubMGs.

A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report

Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder in humans included in the group of Transmissible Spongiform Encephalopathies or prion diseases. The vast majority of sCJD cases are molecularly classified according to the abnormal prion protein (PrPSc) conformations along with polymorphism of codon 129 of the PRNP gene. Recently, a novel human disease, termed "protease-sensitive prionopathy", has been described. This disease shows a distinct clinical and neuropathological phenotype and it is associated to an abnormal prion protein more sensitive to protease digestion. Case presentation: We report the case of a 75-year-old-man who developed a clinical course and presented pathologic lesions compatible with sporadic Creutzfeldt-Jakob disease, and biochemical findings reminiscent of "protease-sensitive prionopathy". Neuropathological examinations revealed spongiform change mainly affecting the cerebral cortex, putamen/globus pallidus and thalamus, accompanied by mild astrocytosis and microgliosis, with slight involvement of the cerebellum. Confluent vacuoles were absent. Diffuse synaptic PrP deposits in these regions were largely removed following proteinase treatment. PrP deposition, as revealed with 3F4 and 1E4 antibodies, was markedly sensitive to pre-treatment with proteinase K. Molecular analysis of PrPSc showed an abnormal prion protein more sensitive to proteinase K digestion, with a five-band pattern of 28, 24, 21, 19, and 16 kDa, and three aglycosylated isoforms of 19, 16 and 6 kDa. This PrPSc was estimated to be 80% susceptible to digestion while the pathogenic prion protein associated with classical forms of sporadic Creutzfeldt-Jakob disease were only 2% (type VV2) and 23% (type MM1) susceptible. No mutations in the PRNP gene were found and genotype for codon 129 was heterozygous methionine/valine. Conclusions: A novel form of human disease with abnormal prion protein sensitive to protease and MV at codon 129 was described. Although clinical signs were compatible with sporadic Creutzfeldt-Jakob disease, the molecular subtype with the abnormal prion protein isoforms showing enhanced protease sensitivity was reminiscent of the "protease-sensitive prionopathy". It remains to be established whether the differences found between the latter and this case are due to the polymorphism at codon 129. Different degrees of proteinase K susceptibility were easily determined with the chemical polymer detection system which could help to detect proteinase-susceptible pathologic prion protein in diseases other than the classical ones.

Pintura e Ironía. La interrogación irónica en la expresión pictórica

Tesis con Mención de Doctor Internacional

El efecto placebo en productos artísticos; el caso de la poesía

[ES] Esta investigación aporta evidencias concluyentes de que existe el efecto placebo en la valoración de un producto artístico, en este caso un poema. Tras la aplicación de diferentes metodologías y testando divergentes poemas, los resultados indican que el nombre del autor influye significativamente en la valoración subjetiva que se hace de la obra. De este modo, esta investigación da respuesta a la pregunta: ¿puede una pieza literaria ser evaluada de manera diferente en función de la persona que firma esa creación? Los resultados de este estudio ciertamente muestran ese efecto: un poema es evaluado de manera significativamente más favorable cuando el que lo lee cree que esa obra está firmada por un gran artista, en contraposición a que ese poema fuera anónimo. O lo que es lo mismo, el nombre de marca condiciona la percepción del producto. Sin embargo, el efecto placebo se debilita cuando el estímulo es evaluado por personas con alta experiencia con el producto. Las implicaciones para el marketing y para el sector editorial son discutidas finalmente.

Tipografía Popular Urbana: Los rótulos del pequeño negocio en el paisaje de Bilbao

419 páginas. 561 fotografías originales.

The role of syllables in sign language production

The aim of the present study was to investigate the functional role of syllables in sign language and how the different phonological combinations influence sign production. Moreover, the influence of age of acquisition was evaluated. Deaf signers (native and non-native) of Catalan Signed Language (LSC) were asked in a picture-sign interference task to sign picture names while ignoring distractor-signs with which they shared two phonological parameters (out of three of the main sign parameters: Location, Movement, and Handshape). The results revealed a different impact of the three phonological combinations. While no effect was observed for the phonological combination Handshape-Location, the combination Handshape-Movement slowed down signing latencies, but only in the non-native group. A facilitatory effect was observed for both groups when pictures and distractors shared Location-Movement. Importantly, linguistic models have considered this phonological combination to be a privileged unit in the composition of signs, as syllables are in spoken languages. Thus, our results support the functional role of syllable units during phonological articulation in sign language production.

Hermenéutica de la astrología greco-romana

[ES] La astrología grecorromana es un código lingüístico con signos (planetas, signos del Zodíaco), y con leyes de interconexión entre ellos (eclíptica, aspectos, casas, límites), y ha de ser valorada no sólo desde la perspectiva de su cientificidad, sino desde la de sus funciones para el individuo y la sociedad: es un conocimiento del futuro singular que afecta a la totalidad de lo implicado, y en la totalidad de sus dimensiones. Suple así el conocimiento de la ciencia antigua, que no fue capaz de incluir ni lo futuro ni lo singular. La astrología integra al individuo en la comunidad, integrándolo previamente en su universo semántico. Cosmos, mundo humano y mundo natural se armonizan totalmente.

Exploring Genetic Factors Involved in Huntington Disease Age of Onset: E2F2 as a New Potential Modifier Gene

Age of onset (AO) of Huntington disease (HD) is mainly determined by the length of the CAG repeat expansion (CAGexp) in exon 1 of the HTT gene. Additional genetic variation has been suggested to contribute to AO, although the mechanism by which it could affect AO is presently unknown. The aim of this study is to explore the contribution of candidate genetic factors to HD AO in order to gain insight into the pathogenic mechanisms underlying this disorder. For that purpose, two AO definitions were used: the earliest age with unequivocal signs of HD (earliest AO or eAO), and the first motor symptoms age (motor AO or mAO). Multiple linear regression analyses were performed between genetic variation within 20 candidate genes and eAO or mAO, using DNA and clinical information of 253 HD patients from REGISTRY project. Gene expression analyses were carried out by RT-qPCR with an independent sample of 35 HD patients from Basque Country Hospitals. We found suggestive association signals between HD eAO and/or mAO and genetic variation within the E2F2, ATF7IP, GRIN2A, GRIN2B, LINC01559, HIP1 and GRIK2 genes. Among them, the most significant was the association between eAO and rs2742976, mapping to the promoter region of E2F2 transcription factor. Furthermore, rs2742976 T allele patient carriers exhibited significantly lower lymphocyte E2F2 gene expression, suggesting a possible implication of E2F2-dependent transcriptional activity in HD pathogenesis. Thus, E2F2 emerges as a new potential HD AO modifier factor.