On Unconditionally Secure Distributed Oblivious Transfer.

This paper is about the Oblivious Transfer in the distributed model proposed by M. Naor and B. Pinkas. In this setting a Sender has n secrets and a Receiver is interested in one of them. During a set up phase, the Sender gives information about the secrets to m Servers. Afterwards, in a recovering phase, the Receiver can compute the secret she wishes by interacting with any k of them. More precisely, from the answers received she computes the secret in which she is interested but she gets no information on the others and, at the same time, any coalition of k − 1 Servers can neither compute any secret nor figure out which one the Receiver has recovered. We present an analysis and new results holding for this model: lower bounds on the resources required to implement such a scheme (i.e., randomness, memory storage, communication complexity); some impossibility results for one-round distributed oblivi- ous transfer protocols; two polynomial-based constructions implementing 1-out-of-n dis- tributed oblivious transfer, which generalize and strengthen the two constructions for 1-out-of-2 given by Naor and Pinkas; as well as new one-round and two-round distributed oblivious transfer protocols, both for threshold and general access structures on the set of Servers, which are optimal with respect to some of the given bounds. Most of these constructions are basically combinatorial in nature

A wave-to-wire model for grid integration studies of oscillating-body wave energy converters

Wave energy converters (WECs) are still at an earlier stage of development when compared to variable renewable energy systems based on wind or solar power. Indeed, only a few WECs have exported power to electric grids until recently. Thus, the development of mathematical models able to represent essential aspects of the system and its connection to the grid becomes fundamental to assess the impact of integrating wave power to grids. This work develops a fully integrated wave-to-wire model, where the electrical model has re-configurable dynamic models of rotary and linear generators (with controllers) to accommodate different types of oscillating-body systems. Such an electrical model is interfaced with the WEC hydrodynamic and mechanical models. A complete wave-to-grid model is presented by integrating the generator system model, an electrical grid interface unit and a network equivalent for the receiving grid in a unified simulation environment with the WEC-Sim, an open-source tool for simulating the dynamic behaviour of WECs. Numerical simulation studies are presented considering different operating conditions for the grid integration of a floating body that is connected to either an hydraulic power take-off system or a direct-drive system.A wave-to-wire model for grid integration studies of oscillating-body wave energy convertersacceptedVersio

Seasonal variability of the HO.RE.CA. food leftovers employed as a feeding substrate for black soldier fly (Hermetia illucens L.) larvae and effects on the rearing performance

The SCALIBUR project (Horizon, 2020) aimed to explore innovative solutions, including the use of black soldier fly larvae, for the bio-urban waste management. This research work describes the evaluation of the variability in water, proteins, fat, ashes, and carbohydrates present in the HO.RE.CA. food leftovers which were withdrawn from a local canteen over a 12-month period and the relationship with (i) the growth parameters of the larvae, (ii) the percentage of substrate reduction and the percentage of frass separated through the mechanical sieve at the end of the rearing process. HO.RE.CA. food leftovers are overall a suitable feeding substrate for larval rearing. Water contained in the HO.RE.CA. food leftovers was sufficient for larval rearing without resorting to further addition. As for water content, a seasonal trend was not observed, on the contrary, it was proved to be totally random. However, high amount of water (>80%) was correlated with higher larval mortality rate. The larval weight was significantly correlated to the amount of protein (r = 0.80; p ≤ 0.001) present in the substrate, and to a lesser extent to the amount of fat (r = 0.43; p ≤ 0.05). The feed conversion rate and bioconversion rate were both in agreement with literature data. The statistical test did not show any significant correlation between the amount of water contained in the initial fresh HO.RE.CA. food leftovers and the percentage of substrate reduction and the percentage of frass separated through the mechanical sieve at the end of the rearing process

Constitutional mismatch repair deficiency (CMMRD) presenting with high-grade glioma, multiple developmental venous anomalies and malformations of cortical development-a multidisciplinary/multicentre approach and neuroimaging clues to clinching the diagnosis

Constitutional mismatch repair deficiency syndrome (CMMRD) is a rare cancer-predisposition syndrome associated with a high risk of developing a spectrum of malignancies in childhood and adolescence, including brain tumours. In this report, we present the case of an 8-year-old boy with acute headache, vomiting and an episode of unconsciousness in whom brain imaging revealed a high-grade glioma (HGG). The possibility of an underlying diagnosis of CMMRD was suspected radiologically on the basis of additional neuroimaging findings, specifically the presence of multiple supratentorial and infratentorial developmental venous anomalies (DVAs) and malformations of cortical development (MCD), namely, heterotopic grey matter. The tumour was debulked and confirmed to be a HGG on histopathology. The suspected diagnosis of CMMRD was confirmed on immunohistochemistry and genetic testing which revealed mutations in PMS2 and MSH6. The combination of a HGG, multiple DVAs and MCD in a paediatric or young adult patient should prompt the neuroradiologist to suggest an underlying diagnosis of CMMRD. A diagnosis of CMMRD has an important treatment and surveillance implications not only for the child but also the family in terms of genetic counselling

'Mutations in LAMB2 associate with albuminuria and Optic Nerve Hypoplasia with Hypopituitarism'

CONTEXT: Mutations in LAMB2, encoding the basement membrane protein, laminin β2, are associated with an autosomal recessive disorder characterized by congenital nephrotic syndrome, ocular abnormalities and neurodevelopmental delay (Pierson Syndrome). CASE DESCRIPTION: This report describes a twelve year old boy with short stature, visual impairment and developmental delay who presented with macroscopic haematuria and albuminuria. He had isolated growth hormone deficiency, optic nerve hypoplasia and a small anterior pituitary with corpus callosum dysgenesis on his cranial MRI, thereby supporting a diagnosis of optic nerve hypoplasia syndrome. Renal histopathology revealed focal segmental glomerulosclerosis. Using next generation sequencing on a targeted gene panel for steroid resistant nephrotic syndrome, compound heterozygous missense mutations were identified in LAMB2 [c.737G>A p.Arg246Gln, c.3982G>C p.Gly1328Arg]. Immunohistochemical analysis revealed reduced glomerular laminin β2 expression compared to control kidney and a thin basement membrane on electron microscopy. Laminin β2 is expressed during pituitary development and Lamb2-/- mice exhibit stunted growth, abnormal neural retinae and here, we show, abnormal parenchyma of the anterior pituitary gland. CONCLUSION: We propose that patients with genetically undefined optic nerve hypoplasia syndrome should be screened for albuminuria and if present, screened for mutations in LAMB2

Janus kinase 1/2 inhibition for the treatment of autoinflammation associated with heterozygous TNFAIP3 mutation

We report a case of autoinflammation caused by a heterozygous variant in TNFAIP3 manifesting as progressive neuroinflammation. Mutant A20 protein failed to control interferon-dependent transcription. We demonstrate excellent therapeutic response to Janus kinase inhibition

Acidosis and Deafness in Patients with Recessive Mutations in FOXI1

Maintenance of the composition of inner ear fluid and regulation of electrolytes and acid-base homeostasis in the collecting duct system of the kidney require an overlapping set of membrane transport proteins regulated by the forkhead transcription factor FOXI1. In two unrelated consanguineous families, we identified three patients with novel homozygous missense mutations in FOXI1 (p.L146F and p.R213P) predicted to affect the highly conserved DNA binding domain. Patients presented with early-onset sensorineural deafness and distal renal tubular acidosis. In cultured cells, the mutations reduced the DNA binding affinity of FOXI1, which hence, failed to adequately activate genes crucial for normal inner ear function and acidbase regulation in the kidney. A substantial proportion of patients with a clinical diagnosis of inherited distal renal tubular acidosis has no identified causative mutations in currently known disease genes. Our data suggest that recessive mutations in FOXI1 can explain the disease in a subset of these patients

Open access simulation toolbox for the grid connection of offshore wind farms using multi-terminal HVDC networks

Decarbonisation of the European electricity system can become dauntingly costly due to transmission and distribution network issues arising from the integration of intermittent renewable generation sources. It is expected that wind energy will be the principal renewable source by 2050 and, as such, a number of initiatives in the academia and in the industry are being carried out to propose solutions to best accommodate the wind resource. This paper presents work carried out by DEMO 1 partners within the EU FP7 project BEST PATHS. A MATLAB/Simulink toolbox consisting of the necessary building blocks for the simulation and integration of offshore wind farms using enabling technologies such as multiterminal high-voltage direct-current grids is presented. To illustrate the toolbox capabilities, a number of system topologies is studied. System performance is assessed and measured against a set of key performance indicators. To ensure knowledge dissemination, the toolbox has been made available as open access in the BEST PATHS project website

Mutated CaV2.1 channels dysregulate CASK/P2X3 signaling in mouse trigeminal sensory neurons of R192Q Cacna1a knock-in mice

Background: ATP-gated P2X3 receptors of sensory ganglion neurons are important transducers of pain as they adapt their expression and function in response to acute and chronic nociceptive signals. The present study investigated the role of calcium/calmodulin-dependent serine protein kinase (CASK) in controlling P2X3 receptor expression and function in trigeminal ganglia from Cacna1a R192Q-mutated knock-in (KI) mice, a genetic model for familial hemiplegic migraine type-1.Results: KI ganglion neurons showed more abundant CASK/P2X3 receptor complex at membrane level, a result that likely originated from gain-of-function effects of R192Q-mutated CaV2.1 channels and downstream enhanced CaMKII activity. The selective CaV2.1 channel blocker \u3c9-Agatoxin IVA and the CaMKII inhibitor KN-93 were sufficient to return CASK/P2X3 co-expression to WT levels. After CASK silencing, P2X3 receptor expression was decreased in both WT and KI ganglia, supporting the role of CASK in P2X3 receptor stabilization. This process was functionally observed as reduced P2X3 receptor currents.Conclusions: We propose that, in trigeminal sensory neurons, the CASK/P2X3 complex has a dynamic nature depending on intracellular calcium and related signaling, that are enhanced in a transgenic mouse model of genetic hemiplegic migraine. \ua9 2013 Gnanasekaran et al.; licensee BioMed Central Ltd