Carta enviada por Juan Alcorta Maiz a Justo Garate en 1981

1 carta (mecanografiada) ; 210x274 mm. Ubicación: Caja 1 - Carpeta 19

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.

^(18)O/^(16)O and D/H studies on the interactions between heated meteoric ground waters and igneous intrusions: Western San Juan Mountains, Colorado and the Isle of Skye, Scotland

Detailed oxygen, hydrogen and carbon isotope studies have been carried out on igneous and metamorphic rocks of the Stony Mountain complex, Colorado, and the Isle of Skye, Scotland, in order to better understand the problems of hydrothermal meteoric water-rock interaction.

The Tertiary Stony Mountain stock (~1.3 km in diameter), is composed of an outer diorite, a main mass of biotite gabbro, and an inner diorite. The entire complex and most of the surrounding country rocks have experienced various degrees of ¹⁸O depletion (up to 10 per mil) due to interaction with heated meteoric waters. The inner diorite apparently formed from a low-¹⁸O magma with δ¹⁸O ≃ +2.5, but most of the isotopic effects are a result of exchange between H₂O and solidified igneous rocks. The low-¹⁸O inner diorite magma was probably produced by massive assimilation and/or melting of hydrothermally altered country rocks. The δ¹⁸O values of the rocks generally increase with increasing grain size, except that quartz typically has δ¹⁸O = +6 to +8, and is more resistant to hydrothermal exchange than any other mineral studied. Based on atom % oxygen, the outer diorites, gabbros, and volcanic rocks exhibit integrated water/rock ratios of 0.3 ± 0.2, 0.15 ± 0.1, and 0.2 ± 0.1, respectively. Locally, water/rock ratios attain values greater than 1.0. Hydrogen isotopic analyses of sericites, chlorites, biotites, and amphiboles range from -117 to -150. δD in biotites varies inversely with Fe/Fe+Mg, as predicted by Suzuoki and Epstein (1974), and positively with elevation, over a range of 600 m. The calculated δD of the mid-to-late-Tertiary meteoric waters is about -100. Carbonate δ¹³C values average -5.5 (PDB), within the generally accepted range for deep-seated carbon.

Almost all the rocks within 4 km of the central Tertiary intrusive complex of Skye are depleted in ¹⁸O. Whole-rock δ¹⁸O values of basalts (-7. 1 to +8.4), Mesozoic shales (-0.6 to + 12.4), and Precambrian sandstones (-6.2 to + 10.8) systematically decrease inward towards the center of the complex. The Cuillin gabbro may have formed from a ¹⁸O-depleted magma (depleted by about 2 per mil); δ¹⁸O of plagioclase (-7.1 to + 2.5) and pyroxene (-0.5 to + 3.2) decrease outward toward the margins of the pluton. The Red Hills epigranite plutons have δ¹⁸O quartz (-2.7 to + 7.6) and feldspar (-6.7 to + 6.0) that suggest about 3/4 of the exchange took place at subsolidus temperatures; profound disequilibrium quartz-feldspar fractionations (up to 12) are characteristic. The early epigranites were intruded as low-¹⁸O melts (depletions of up to 3 per mil) with δ¹⁸O of the primary, igneous quartz decreasing progressively with time. The Southern Porphyritic Epigranite was apparently intruded as a low-¹⁸O magma with δ¹⁸O ≃ -2.6. A good correlation exists between grain size and δ¹⁸O for the unique, high-¹⁸O Beinn an Dubhaich granite which intrudes limestone having a δ¹⁸O range of +0.5 to +20.8, and δ¹³C of -4.9 to -1.0. The δD values of sericites (-104 to -107), and amphiboles, chlorites, and biotites (-105 to -128) from the igneous rocks , indicate that Eocene surface waters at Skye had δD ≃ -90. The average water/rock ratio for the Skye hydrothermal system is approximately one; at least 2000 km³ of heated meteoric waters were cycled through these rocks.

Thus these detailed isotopic studies of two widely separated areas indicate that (1) ¹⁸O-depleted magmas are commonly produced in volcanic terranes invaded by epizonal intrusions; (2) most of the ¹⁸O-depletion in such areas are a result of subsolidus exchange (particularly of feldspars); however correlation of δ¹⁸O with grain size is generally preserved only for systems that have undergone relatively minor meteoric hydrothermal exchange; (3) feldspar and calcite are the minerals mos t susceptible to oxygen isotopic exchange, whereas quartz is very resistant to oxygen isotope exchange; biotite, magnetite, and pyroxene have intermediate susceptibilities; and (4) basaltic country rocks are much more permeable to the hydrothermal convective system than shale, sandstone, or the crystalline basement complex.

Ecosystem study Altenwoerth: impacts of a hydroelectric power-station on the River Danube in Austria

The aim of this article is to briefly describe the effects of the Altenwoerth Barrage, on the River Danube, on some physical variables and their consequent effects on water chemistry and the biota of the river. The methods used for biological sampling are summarised, especially those used in the limnological part of the study, and the macroinvertebrate and fish fauna listed. Comparisons are then made between the impounded section of river immediately above the dam and two unimpounded free-flowing sections of the river. Further developments on the Danube are considered.

The distribution of native and introduced species of crayfish in Austria

This account concentrates on the six species of crayfish found in Austria, and the current state of knowledge on their distribution and laws affecting conservation. In general the occurrence and distribution of crayfish in Austria is poorly known, although information obtained by researchers and the general public, after careful checking, is increasing. Three native crayfish species occur in Austria: Austropotamobius torrentium which is relatively widespread, A. pallipes with a restricted distribution, and Astacus astacus which is widespread. Three species of non-native (alien) crayfish have been recorded from a total of 158 localities in Austria. They are Astacus leptodactylus from eastern Europe, and two Nearctic species: Pacifastacus leniusculus and Orconectes limosus. The introduction of alien species causes considerable problems as they act as vectors of crayfish plague and are able to outcompete native species by higher reproductive capacities.

Análisis del mercado eléctrico EPEX SPOT (Austria, Alemania, Suiza, Francia)

El objetivo de este trabajo es analizar el mercado EPEX Spot, con el fin de ver si mediante la integración de mercados se consigue converger los precios de la electricidad de Francia, Alemania, Suiza y Austria. En primer lugar se hará una breve descripción del proceso de liberalización llevado a cabo por los países que forman este mercado. A continuación, se introducirá EPEX Spot explicando el funcionamiento y los tipos de mercados que contiene. En la tercera sección se definirá el proceso de la armonización del mercado europeo. Por último, se hará un análisis más exhaustivo de la estructura de mercado de cada país y se examinará la evolución de los precios.

Comparative drift studies on streams in lower Austria (Flysch, gneiss and chalk formations) [Translation from: Schweizerische Zeitschrift fur Hydrobiologie 30 138-185, 1968.]

Seasonal changes and flooding have an extraordinarily great influence on the drift of organisms. The free water space plays the main part in the provision of food for some fish (Salmo trutta - trout): drift and content of the stomach are balanced here (Simuliidae): whereas others (Thymallus vulgaris) only selectively chose certain animals living at the bottom (molluscs). The total drift, drift of organisms and drift of organic material and minerals, plays a main role in the rate of production in streams. Besides the biology of the organisms living on the river bed, also the geological and hydrographical situation of the area plays a very important role for the composition of the drift. During the years 1964-1966 three streams in the characteristical geological formations flysch, gneiss and chalk of lower Austria were studied in regard to their drift. The Tulln (above St. Christopen), the Krems (above Senftenberg) and the Schwarza (above Hirschwang) seemed to be ideal for this comparative study because they are easy to reach. After summarising the hydrography and chemistry of examined rivers, the author examines the relationship between water level and total drift and the stratification of the total drift before analysing the drift of living organisms. Also considered are seasonal changes of drift of organisms and drift of exuviae.

El pintor navarro Juan Ochoa de Arín (1600-1652) y su producción en Gipuzkoa

Investigación realizada dentro del Programa de Formación y Perfeccionamiento de Personal Investigador del Gobierno Vasco (2010-2013).

Juan Antonio de Zamacola: vida, obra y concepción esteticomusical

Tesis doctoral originalmente defendida en la Universidad de Deusto el 29-09-2000

Diseño e implementación de una herramienta web para el análisis y ayuda para la toma de decisiones de tecnologías de acceso.

[EN]Nowadays the use of web applications is a routine not only for companies but also for anyone interested in them. Thus, this market has risen hugely since the introduction of The Internet in our daily lives. Everyone has experienced the moment when you have to choose an access service and you do not know which one to select. At that moment, it is when this web application comes into action. It provides a useful interface in order to choose between access services as well as an analysis tool for the different access technologies in the market. Written in Java language, this web application is as simple as it can be, offering a complete interface that meets the needs of everyone, from the people at home to the largest company.

Carta enviada por Juan Bueso Roda a Justo Garate en 1939

1 carta (manuscrita) ; 225x330 mm

¿Del estilo paleolítico a la cronología contemporánea?: Una (revisión) nueva versión del arte parietal de la cueva de El Becerral (La Gándara, Cantabria)

Homenaje a Ignacio Barandiarán Maestu / coord. por Javier Fernández Eraso, Juan Santos Yanguas

Las industrias prehistóricas del yacimiento de San Juan (Castro Urdiales, Cantabria) y su contextualización cronológica y cultural

[ES] El análisis directo de los ajuares lítico y óseo que se conservan del yacimiento de San juan, nos induce a plantear una hipótesis principal, según la cual la ocupación prehistórica del depósito debió producirse fundamentalmente durante el Aziliense, aunque no puede descartarse un origen anterior, en las últimas fases del Magdaleniense.

Ictiólogos de la Argentina: Juan Carlos Chebez

This series will include all those people who, by means of their contributions, great and small, played a part in the consolidation of ichthyology in Argentina. The general plan of this work consists of individual factsheets containing a list of works by each author, along with reference bibliography and, whenever possible, personal pictures and additional material. The datasheets will be published primarily in chronological order, although this is subject to change by the availability of materials for successive editions. This work represents another approach for the recovery and revalorization of those who set the foundations of Argentine ichthyology while in diverse historical circumstances. I expect this to be the beginning of a major work that achieves the description of such a significant part of the history of natural sciences in Argentina.