1,823 research outputs found
ASM variants in the spotlight:A structure-based atlas for unraveling pathogenic mechanisms in lysosomal acid sphingomyelinase
No full textLysosomal acid sphingomyelinase (ASM), a critical enzyme in lipid metabolism encoded by the SMPD1 gene, plays a crucial role in sphingomyelin hydrolysis in lysosomes. ASM deficiency leads to acid sphingomyelinase deficiency, a rare genetic disorder with diverse clinical manifestations, and the protein can be found mutated in other diseases. We employed a structure-based framework to comprehensively understand the functional implications of ASM variants, integrating pathogenicity predictions with molecular insights derived from a molecular dynamics simulation in a lysosomal membrane environment. Our analysis, encompassing over 400 variants, establishes a structural atlas of missense variants of lysosomal ASM, associating mechanistic indicators with pathogenic potential. Our study highlights variants that influence structural stability or exert local and long-range effects at functional sites. To validate our predictions, we compared them to available experimental data on residual catalytic activity in 135 ASM variants. Notably, our findings also suggest applications of the resulting data for identifying cases suited for enzyme replacement therapy. This comprehensive approach enhances the understanding of ASM variants and provides valuable insights for potential therapeutic interventions.</p
Changes in the insect emergence at the water–air interface in response to fish density manipulation in high altitude lakes.
No full textEmerging insects are important nutrient vectors for lake-to-land linkages, but they are affected by fish predation. We manipulated the fish density in four high altitude lakes in connection with a brook trout eradication project. We used a paired benthic and surface funnel trap design to test whether fish density affects abundance, size, and proportion of aquatic insects reaching the surface in the pelagic zone. The study was complemented with benthic macroinvertebrates and fish diet data. Due to sample size, we focused on midge pupae, which rely on water surface tension (water–air interface) to emerge. Conspicuous insects emerging along the shoreline (water–land interface) were rarely collected in the traps, even if they rapidly recovered through the eradication. There was a positive relationship between fish density and midge abundance, and negative relationship for size and proportion reaching the surface. The increase in the emergence of midges in the presence of fish supports several other studies, but we have provided evidence that this pattern exists in conjunction with a size reduction even when fish consume a substantial amount of midges. The inclusion of small Diptera emergence at the water–air interface is a likely keystone in understanding fish impact on insect subsidy
TUTELA DEL LAVORO E LIBERTA' D'IMPRESA NEI PROCESSI DI ESTERNALIZZAZIONE
No full textL’elaborato analizza le conseguenze lavoristiche della successione fra imprenditori, muovendo da una ricognizione delle varie tipologie di esternalizzazione con le relative esigenze e principali criticità.
L’indagine si concentra in primo luogo sul trasferimento d’azienda, esaminando la normativa e la giurisprudenza europee per passare poi alla disciplina di diritto interno, alle procedure sindacali e a uno specifico focus sul trasferimento delle aziende in crisi.
Successivamente l’autore si sofferma sull’appalto, prendendone in particolare considerazione gli indici di genuinità, i criteri di distinzione dalla somministrazione illecita di manodopera e la tutela delle maestranze in caso di avvicendamento fra imprese.
Da ultimo, la ricerca approfondisce le c.d. “clausole sociali”, sia di prima che di seconda generazione, valutandone la compatibilità con il diritto eurounitario e con la costituzione nonché riflettendo sui possibili rimedi in caso di loro violazione.The author analyzes the labour consequences of the succession between entrepreneurs, starting from a recognition of the various types of outsourcing with the related needs and main critical issues.
The survey focuses primarily on the transfer of businesses, examining European legislation and case-law and then moving on to internal legislation, trade union procedures and a specific focus on the transfer of companies in crisis.
The author then dwells on the contract, taking into account in particular the indications of authenticity, the criteria of distinction from the illicit administration of labour and the protection of workers in the event of turnover between companies.
Finally, the research deepens the "social clauses", both first and second generation, assessing their compatibility with European law and with the constitution and reflecting on possible remedies in case of their violation
NDP52 GtoE mutation
No full textCollection of simulations and free energy calculations used in the publication:
Characterization of a natural variant of human NDP52 and its functional consequences on mitophagy.
Di Rita A, Angelini DF, Maiorino T, Caputo V, Cascella R, Kumar M, Tiberti M, Lambrughi M, Wesch N, Löhr F, Dötsch V, Carinci M, D'Acunzo P, Chiurchiù V, Papaleo E, Rogov VV, Giardina E, Battistini L, Strappazzon F.
Cell Death Differ. 2021, 28(8):2499-2516. doi: 10.1038/s41418-021-00766-3
Predation by introduced fish can magnify the terrestrial arthropod subsidies in mountain lakes
No full textA portion of the terrestrial subsidies to lentic habitats consists of arthropods. In high mountain, originally fishless lakes, terrestrial arthropods are an important seasonal food resource for introduced fish. Here we investigate how brook trout Salvelinus fontinalis can alter the input of terrestrial arthropods in ten high mountain lakes contrasting for their stocking history (with and without fish, or manipulated for fish eradication). We used a food consumption model to calculate the minimum and maximum number/biomass of arthropods consumed by fish and we found that they can exceed several folds the number/biomass of arthropods sinking into the lakes, at least under the metabolic rates expected for fish for most of the summer. We interpret this result as an indirect indication that arthropods usually cannot overcome the surface tension at the lake surface and that fish can work as a vector across the water-air interface. We infer that pathways for dead and alive arthropods to leave the lakes do exist and fish can transfer into the water column many arthropods whose fate was leaving the lakes, which may have overlooked ecological and conservation implications.The accepted manuscript in pdf format is listed with the files at the bottom of this page. The presentation of the authors' names and (or) special characters in the title of the manuscript may differ slightly between what is listed on this page and what is listed in the pdf file of the accepted manuscript; that in the pdf file of the accepted manuscript is what was submitted by the author
Ultra Low Carbon Vehicles: New Parameters for Automotive Design
No full textAs the influence of vehicle emissions on our environment has become better understood, the UK government has recently placed urgent emphasis on the implementation of low carbon
technologies in the automotive industry through: the UK Low Carbon Industrial Strategy. The overall objective is to offer big incentives to consumers and support for the development of infrastructure and engineering solutions. This scheme however does not consider how the development of functional and experiential user value might drive consumer demand, contributing to the adoption of low carbon vehicles (LCVs) in the mass market.
With the emergence of the North East of England as the UK’s first specialised region for the development of ultra-low carbon vehicles (ULCVs), ONE North East, as a development agency for the region's economic and business development, and Northumbria University Ideas-lab have supported a project to facilitate innovation through the collaboration of technology, research and development (R&D) and business. The High Value Low Carbon (HVLC) project aims to envisage
new user value made possible by the integration of low carbon vehicle platforms with new process and network technologies. The HVLC consortium represents vehicle manufacturers and their suppliers as well as technology based companies and through an ongoing process of design concept generation the project offers a hub for innovation led enterprise.
Whilst new technological developments in areas such as power generation, nano materials, hydrogen fuel cells, printed electronics and networked communications will all impact on future automotive design, the mass adoption of low carbon technologies represents a paradigm shift for the motorist. This paper aims to describe how the mapping of new parameters will lead to new transport scenarios that will create the space for new collaborative research on user experiences supported by innovative technologies and related services
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Matteo Maria Boiardo (1441-1494)
No full textMatteo Maria Boiardo – contemporary of Sir Thomas Malory and forerunner of Shakespeare, Spenser, Milton, Cervantes, Tolkien, and C.S. Lewis – is best known as the author of the Orlando Innamorato [Orlando in Love], a poem in ottava rima organically merging Carolingian epic and Arthurian romance. He was also a lyric and pastoral poet, playwright, and translator of classical texts into the Italian vernacular. Matteo was active in Italy during the years of 1463 throughout 1494, the year of his death
Uno sguardo all'Est. Lavoro e diritti fondamentali in Russia e negli altri Paesi della Comunità di Stati indipendenti
No full textNel contributo l'autore, dopo aver esaminato i diritti fondamentali del lavoro presenti nelle Costituzioni dei Paesi della Comunità di Stati indipendenti, si sofferma sul diritto del lavoro russo. La trattazione, a carattere diacronico, abbraccia i rami principali del diritto del lavoro: il rapporto individuale, le relazioni collettive, il mercato del lavoro. L'autore rileva una sorprendente continuità nelle linee essenziali del diritto del lavoro russo, pur nel susseguirsi di sistemi socioeconomici completamente diversi. Egli conclude che il diritto del lavoro russo appartiene saldamente alla matrice europea della materia.The contribution of the author starts by examining fundamental labour rights recognised by the Constitutions of the Countries of the Community of Independent States. Then the author focuses upon Russian Labour Law, going through the history of the main branches of the discipline: the individual relationship, industrial relations, the labour market. The article finds a surprising continuity in the essential lines of Russian Labour Law, in spite of the completely different socio-economic systems that were experimented in Russia during the twentieth century. The author concludes that Russian Labour Law clearly belongs to the European matrix of the discipline
Leveling the curve of Spee: comparison between continuous archwire treatment and Invisalign system: a retrospective study
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