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Loss of nuclear TDP-43 is a hallmark of neurodegeneration in TDP-43 proteinopathies, including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). TDP-43 mislocalization results in cryptic splicing and polyadenylation of pre–messenger RNAs (pre-mRNAs) encoding stathmin-2 (also known as SCG10), a protein that is required for axonal regeneration. We found that TDP-43 binding to a GU-rich region sterically blocked recognition of the cryptic 3′ splice site in STMN2 pre-mRNA. Targeting dCasRx or antisense oligonucleotides (ASOs) suppressed cryptic splicing, which restored axonal regeneration and stathmin-2–dependent lysosome trafficking in TDP-43–deficient human motor neurons. In mice that were gene-edited to contain human STMN2 cryptic splice-polyadenylation sequences, ASO injection into cerebral spinal fluid successfully corrected Stmn2 pre-mRNA misprocessing and restored stathmin-2 expression levels independently of TDP-43 binding.

This paper proposes an approach for analyzing the benefits that partial-power-processing-based converters can bring to fully electric maritime applications. With the aim of making the system modular and scalable to different powers/energies, series-connected partial power converters are proposed. Serializing these converters entails significant overvoltage issues, and this paper tackles them for one series-connected module failure case. A reliability analysis has been carried out considering that the components of the battery system follow an independent and identical distribution in terms of failure probability. Furthermore, a redundancy factor has been added to allow a certain failure rate in what is known as a fault-tolerant system. Finally, to demonstrate the high efficiency of partial power converters, a 3 kW prototype is tested at different working points that model the charging process of a battery. The experimental results show a peak efficiency of 99.36%.

[EU] Gaur egun ezagun zaizkigun sehaska-kantak ahozko tradizioaren transmisioari esker ditugu gure artean, baina transmisio hori hiltzear dago. Lan honetan, ahozko tradizioa eta sehaska-kantei buruz adituek diotena ikertu eta iritziak alderatu dira. Adituen iritziak osatzeko asmoz, sehaska-kanten definizio berri bat proposatu da. Behin hau eginda, eskoletan sehaska-kantek duten presentzia, erabilera eta funtzioa aztertu dira galdetegi eta bakarkako elkarrizketen bidez. Jasotako emaitzekin, behar bat identifikatu da, hots, irakasleek sehaska-kantak erabiltzeko duten material falta. Arazo horri erantzun bat emateko intentzioz, sehaska-kantak erabiltzeko informazioa eta materiala eskaini da gida eta web orri baten bidez, ahozko tradizioaren txingarrak luzaroan piztuta jarraitzeko helburuarekin. [ES] Las canciones de cuna que hoy nos son familiares las tenemos entre nosotros gracias a la transmisión de la tradición oral, pero esa transmisión está a punto de morir. En este trabajo se ha investigado la tradición oral y se han contrastado las opiniones de distintos expertos sobre las canciones de cuna. Con el fin de completar las opiniones de los expertos, se ha propuesto una nueva definición de las canciones de cuna. Una vez realizado esto, se ha analizado la presencia, uso y función de las canciones de cuna en las escuelas a través de cuestionarios y entrevistas individuales. Con los resultados obtenidos se ha identificado una necesidad, la falta de material del profesorado para utilizar las canciones de cuna en el aula. Con la intención de dar una respuesta a este problema, se ha ofrecido información y material para el uso de canciones de cuna a través de una guía y una página web, con el objetivo de seguir mucho tiempo con la brasa de las tradición oral encendida. [FR] Les berceuses que nous connaissons aujourd'hui sont le résultat de la transmission de notre tradition orale, mais cette transmission est sur le point de s’éteindre. Ce travail s’appuie sur l’état des lieux de la question ainsi que sur la comparaison des points de vue des experts au sujet de la tradition orale et des berceuses. Afin de compléter ces opinions, une nouvelle définition des berceuses est proposée. Ensuite, la présence, l'utilisation ainsi que la fonction des berceuses dans les écoles sont examinées au moyen de questionnaires et d'entretiens individuels. Grâce aux résultats obtenus, un besoin a été identifié, à savoir le manque de matériel permettant aux enseignant.e.s d'utiliser les berceuses. Dans le but d’apporter une réponse à ce manque, des informations et du matériel sur l'utilisation des berceuses est fournis à travers un guide et un site Internet de manière à entretenir encore longtemps les braises de la tradition orale.

The human mRNA most affected by TDP-43 loss-of-function is transcribed from theSTMN2gene and encodes stathmin-2 (also known as SCG10), whose loss is a neurodegenerative disease hallmark. Here using multiplein vivoapproaches, including transient antisense oligonucleotide (ASO)-mediated suppression, chronic shRNA-mediated depletion in aging mice, and germline deletion, we establish stathmin-2 to be essential for acquisition and maintenance of neurofilament-dependent structuring of axoplasm critical for maintaining diameter and conduction velocity of large-myelinated axons. Sustained stathmin-2 loss from an otherwise mature adult nervous system is demonstrated over a time course of eight months to initiate and drive motor neuron disease that includes 1) shrinkage in inter-neurofilament spacing that is required to produce a three-dimensional space filling array that defines axonal caliber, 2) collapse of mature axonal caliber with tearing of outer myelin layers, 3) reduced conduction velocity, 4) progressive motor and sensory deficits (including reduction of the pain transducing neuropeptide CGRP), and 5) muscle denervation. Demonstration that chronic stathmin-2 reduction is itself sufficient to trigger motor neuron disease reinforces restoration of stathmin-2 as an attractive therapeutic approach for TDP-43-dependent neurodegeneration, including the fatal adult motor neuron disease ALS.

[EU] Maizterraren subrogazioaren azterketa indarrean dagoen Hiri-errentamenduen Legearen eta aurreko lege testuen arauetan. Subrogazioa maizterraren senideek errentamendu-kontratuaren barruan haren posizio juridikoan jarri ahal izateko duten eskubidea da, maizterraren eskubideak eta betebeharrak bereganatuz, betiere lege horietan araututako supostuen barnean. [EN] Analysis of the subrogation of the current and previous urban leasing law. Subrogation is understood as the right of the tenant's relatives to be able to put themselves in their position within the lease, acquiring its rights and obligations, as long as the situations regulated in said laws concur. [ES] Análisis de la subrogación de la persona arrendataria en la vigente Ley de Arrendamientos Urbanos y anteriores textos reguladores. La subrogación se entiende como el derecho de los familiares del arrendatario para colocarse en su posición jurídica dentro del contrato de arrendamiento, adquiriendo los derechos y obligaciones de éste, siempre y cuando concurran los supuestos de hecho contenidos en dichas leyes.

The sharing economy has led to a profound transformation of the tourism sector over the last decade: it has revolutionised the tourist accommodation sector, directly affecting residential markets in destination cities. This paper presents a bibliometric analysis of the research published in Web of Science regarding the sharing economy, tourism and the housing market. 147 studies dealing directly or indirectly with these topics are identified and a bibliometric analysis carried out to determine relationships between the topics and other indicators to measure the breadth of the research. Following this, a deeper content analysis is completed to qualify the most relevant topics of study, supported by data mapping techniques which allow a more immediate visualisation of the subject under study. A systematic review is carried out with a two-fold objective: to analyse and classify the evolution of academic research on this topic in recent years by developing a bibliometric analysis and, in order to enrich our contribution, to identify the main areas of study addressed around this topic. To do this, we analyse the content of the most relevant research papers, classifying trends and subareas of study.

16 pages, 6 figures, 3 tables, supplementary data https://doi.org/10.1016/j.pocean.2021.102659.-- Code and data availability: The experimental protocol in this paper has no code associated with it. Forcing data from CMIP5 used for the protocol, and the FishMIP model outputs presented in this paper are available on the ISIMIP servers (https://www.isimip.org/), Climate change is warming the ocean and impacting lower trophic level (LTL) organisms. Marine ecosystem models can provide estimates of how these changes will propagate to larger animals and impact societal services such as fisheries, but at present these estimates vary widely. A better understanding of what drives this inter-model variation will improve our ability to project fisheries and other ecosystem services into the future, while also helping to identify uncertainties in process understanding. Here, we explore the mechanisms that underlie the diversity of responses to changes in temperature and LTLs in eight global marine ecosystem models from the Fisheries and Marine Ecosystem Model Intercomparison Project (FishMIP). Temperature and LTL impacts on total consumer biomass and ecosystem structure (defined as the relative change of small and large organism biomass) were isolated using a comparative experimental protocol. Total model biomass varied between −35% to +3% in response to warming, and -17% to +15% in response to LTL changes. There was little consensus about the spatial redistribution of biomass or changes in the balance between small and large organisms (ecosystem structure) in response to warming, an LTL impacts on total consumer biomass varied depending on the choice of LTL forcing terms. Overall, climate change impacts on consumer biomass and ecosystem structure are well approximated by the sum of temperature and LTL impacts, indicating an absence of nonlinear interaction between the models’ drivers. Our results highlight a lack of theoretical clarity about how to represent fundamental ecological mechanisms, most importantly how temperature impacts scale from individual to ecosystem level, and the need to better understand the two-way coupling between LTL organisms and consumers. We finish by identifying future research needs to strengthen global marine ecosystem modelling and improve projections of climate change impacts, JDE was funded by Australian Research Council Discovery Projects DP150102656 and DP190102293. MC, JS, NB and OM received financial support by the European Union’s Horizon 2020 research and innovation programme under grant agreement No 817578 (Triatlas project). CH received funding from the Open Philanthropy Project. NB and OM also acknowledge the support of the French ANR project CIGOEF (grant ANR-17-CE32-0008-01). DPT acknowledges funding from the ISI-MIP project to support a workshop on this topic, and the Jarislowsky Foundation. EDG received funding from the European Research Council (ERC) under the European Union’s Horizon 2020 research and innovation programme (grant agreement No 682602, BIGSEA). RFH was funded by the Spanish Ministry of Science, Innovation and Universities through the Acciones de Programación Conjunta Internacional (PCIN-2017-115). MC acknowledges the ‘Severo Ochoa Centre of Excellence’ accreditation (CEX2019-000928-S) to the Institute of Marine Science (ICM-CSIC). TDE acknowledges funding from the ISIMIP project to support a workshop on this topic and the Fisheries and Oceans Canada Atlantic Fisheries Fund. All authors declare no conflict of interest with respect to this study. JAFS received funding from the European Union’s Horizon 2020 FutureMARES project (#869300).