Influence on present-day coastal dynamics and evolution of a relict subaqueous delta lobe: Sol de Riu lobe, Ebro Delta. Continental Shelf Research, 74: 94-104. doi:10.1016/j.csr.2013.11.021

We used high-resolution swath-bathymetry data to characterise the morphology of the abandoned subaqueous Sol de Riu delta lobe in the Ebro Delta, Western Mediterranean Sea. This study aims to assess the influence of an abandoned delta lobe on present-day coastal dynamics in a micro-tidal environment. Detailed mapping of the relict Sol de Riu lobe also showed a set of bedforms interpreted as footprints of human activities: seasonal V-shaped depressions on the middle shoreface due to boat anchoring and old trawling marks between 16 and 18 m water depth. Estimations of the mobility of bottom sediment showed that the shallowest shoreface (i.e. less than 7 m depth) is the most dynamic part of the relict lobe, while the middle shoreface experienced significant morphological changes since the lobe was abandoned. The deepest shoreface (i.e. water depth in excess of 15 m), which corresponds to the front of the lobe, is defined by a very small potential for morphological change. Simulations showed that while the relict lobe does not significantly affect the typical short period waves (Tp ≈4 s) in the study area, it does interfere with the most energetic wave conditions (Tp ≥ 7 s) acting as a shoal leading to the concentration of wave energy along the shoreline northwest of the lobe. The consequence of such modification of the high-energy wave propagation pattern by the relict lobe is an alteration of the wave-induced littoral sediment dynamics with respect to a situation without the lobe.

RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of machado-joseph disease.

Machado-Joseph disease or Spinocerebellar ataxia type 3 is a progressive fatal neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3. Recent studies demonstrate that RNA interference is a promising approach for the treatment of Machado-Joseph disease. However, whether gene silencing at an early time-point is able to prevent the appearance of motor behavior deficits typical of the disease when initiated before onset of the disease had not been explored. Here, using a lentiviral-mediated allele-specific silencing of mutant ataxin-3 in an early pre-symptomatic cerebellar mouse model of Machado-Joseph disease we show that this strategy hampers the development of the motor and neuropathological phenotypic characteristics of the disease. At the histological level, the RNA-specific silencing of mutant ataxin-3 decreased formation of mutant ataxin-3 aggregates, preserved Purkinje cell morphology and expression of neuronal markers while reducing cell death. Importantly, gene silencing prevented the development of impairments in balance, motor coordination, gait and hyperactivity observed in control mice. These data support the therapeutic potential of RNA interference for Machado-Joseph disease and constitute a proof of principle of the beneficial effects of early allele-specific silencing for therapy of this disease.

A biophysical model of atrial fibrillation ablation: what can a surgeon learn from a computer model?

AIMS: Surgical ablation procedures for treating atrial fibrillation have been shown to be highly successful. However, the ideal ablation pattern still remains to be determined. This article reports on a systematic study of the effectiveness of the performance of different ablation line patterns. METHODS AND RESULTS: This study of ablation line patterns was performed in a biophysical model of human atria by combining basic lines: (i) in the right atrium: isthmus line, line between vena cavae and appendage line and (ii) in the left atrium: several versions of pulmonary vein isolation, connection of pulmonary veins, isthmus line, and appendage line. Success rates and the presence of residual atrial flutter were documented. Basic patterns yielded conversion rates of only 10-25 and 10-55% in the right and the left atria, respectively. The best result for pulmonary vein isolation was obtained when a single closed line encompassed all veins (55%). Combination of lines in the right/left atrium only led to a success rate of 65/80%. Higher rates, up to 90-100%, could be obtained if right and left lines were combined. The inclusion of a left isthmus line was found to be essential for avoiding uncommon left atrial flutter. CONCLUSION: Some patterns studied achieved a high conversion rate, although using a smaller number of lines than those of the Maze III procedure. The biophysical atrial model is shown to be effective in the search for promising alternative ablation strategies.

Triggering of Bcl-2-related pathway is associated with apoptosis of photoreceptors in Rpe65-/- mouse model of Leber's congenital amaurosis.

Mutations in RPE65 protein is characterized by the loss of photoreceptors, although the molecular pathways triggering retinal cell death remain largely unresolved. The role of the Bcl-2 family of proteins in retinal degeneration is still controversial. However, alteration in Bcl-2-related proteins has been observed in several models of retinal injury. In particular, Bax has been suggested to play a crucial role in apoptotic pathways in murine glaucoma model as well as in retinal detachment-associated cell death. We demonstrated that Bcl-2-related signaling pathway is involved in Rpe65-dependent apoptosis of photoreceptors during development of the disease. Pro-apoptotic Bax alpha and beta isoforms were upregulated in diseased retina. This was associated with a progressive reduction of anti-apoptotic Bcl-2, reflecting imbalanced Bcl-2/Bax ratio as the disease progresses. Moreover, specific translocation of Bax beta from cytosol to mitochondria was observed in Rpe65-deficient retina. This correlated with the initiation of photoreceptor cell loss at 4 months of age, and further increased during disease development. Altogether, these data suggest that Bcl-2-apoptotic pathway plays a crucial role in Leber's congenital amaurosis disease. They further highlight a new regulatory mechanism of Bax-dependent apoptosis based on regulated expression and activation of specific isoforms of this protein.

Auguste Ott on Commercial Crises and Distributive Justice: An Early Input-Output Scheme

In 1851 the French Social economist Auguste Ott discussed the problem of gluts and commercial crises, together with the issue of distributive justice between workers in co-operative societies. He did so by means of a 'simple reproduction scheme' sharing some features with modern intersectoral transactions tables, in particular in terms of their graphical representation. This paper presents Ott's theory of crises (which was based on the disappointment of expectations) and the context of his model, and discusses its peculiarities, supplying a new piece for the reconstruction of the prehistory of input-output analysis.