Alpha-1 antitrypsin deficiency: A conformational disease associated with lung and liver manifestations

Alpha-1 antitrypsin (A1AT) is a serine anti-protease produced chiefly by the liver. A1AT deficiency is a genetic disorder characterized by serum levels of less than 11 μmol/L and is associated with liver and lung manifestations. The liver disease, which occurs in up to 15% of A1AT-deficient individuals, is a result of toxic gain-of-function mutations in the A1AT gene, which cause the A1AT protein to fold aberrantly and accumulate in the endoplasmic reticulum of hepatocytes. The lung disease is associated with loss-of-function, specifically decreased anti-protease protection on the airway epithelial surface. The so-called 'Z' mutation in A1AT deficiency encodes a glutamic acid-to-lysine substitution at position 342 in A1AT and is the most common A1AT allele associated with disease. Here we review the current understanding of the molecular pathogenesis of A1AT deficiency and the best clinical management protocols. © Springer Science+Business Media B.V. 2008.

Indicadores de sostenibilidad ambiental en el periurbano de la ciudad de Mar del Plata, Argentina

Los indicadores de sostenibilidad conforman herramientas útiles para la toma de decisiones. Las ciudades latinoamericanas, y especialmente las áreas de expansión sin planificación adecuada, enfrentan desafíos cada vez mayores para revertir problemáticas que amenazan su sostenibilidad. El presente trabajo evalúa de manera preliminar, la sostenibilidad ambiental del periurbano de Mar del Plata (Argentina) tomando como referencia algunos de los indicadores propuestos por el modelo del Banco Interamericano de Desarrollo en la Iniciativa Ciudades Emergentes y Sostenibles. Se construyó un índice sintético (Índice de Sostenibilidad Ambiental, ISA) que integra trece indicadores agrupados en ocho temas. Las situaciones más críticas (ISA: 0,45-0,558) se identifican fundamentalmente en zonas en las que se desarrollan actividades rurales y en las que se localizan asentamientos de carácter precario. El estudio realizado profundiza en el conocimiento de la dimensión ambiental de la sostenibilidad, enfatizando en el análisis de los contrastes internos del periurbano marplatense.

“The new Cagliostro”: Onofroff, Rubén Darío and his story "La esfinge"

The article retraces the career of the famous hypnotist Onofroff and particularly his visit to Buenos Aires, where his activity, in 1895, provoked many discussions and some public embarrassment. In this context Darío, using a pseudonym, published his crónica “La esfinge”. We reproduce “La esfinge”, transcribing the original text of La Nación, with annotations identifying the literary and theosophical sources Darío used when he wrote it.

Assignment methods for incidence calculus

Incidence calculus is a mechanism for probabilistic reasoning in which sets of possible worlds, called incidences, are associated with axioms, and probabilities are then associated with these sets. Inference rules are used to deduce bounds on the incidence of formulae which are not axioms, and bounds for the probability of such a formula can then be obtained. In practice an assignment of probabilities directly to axioms may be given, and it is then necessary to find an assignment of incidence which will reproduce these probabilities. We show that this task of assigning incidences can be viewed as a tree searching problem, and two techniques for performing this research are discussed. One of these is a new proposal involving a depth first search, while the other incorporates a random element. A Prolog implementation of these methods has been developed. The two approaches are compared for efficiency and the significance of their results are discussed. Finally we discuss a new proposal for applying techniques from linear programming to incidence calculus.

Bimolecular rate constants for diffusion in ionic liquids

The temperature dependence of the bimolecular rate constants for a diffusion controlled reaction involving neutral reactants have been directly determined in five commonly used ionic liquids over the temperature range 5-70 degreesC.

Hydrogen abstraction from ionic liquids by benzophenone triplet excited states

The activation energy for hydrogen abstraction from imidazolium-based ionic liquids is significantly higher than that observed in conventional solvents.