Prospects for a Big Data History of Music

This position paper sets out the possibility of a musicology based on the analysis of musical-bibliographical metadata as Big Data. It outlines the work underway, as part of the AHRC-funded project A Big Data History of Music, to align seven major datasets of musical-bibliographical metadata. After discussing some of the technical challenges of data alignment, it suggests how analysis and visualization of this data might transform musicological understandings of cultural transmission and canon formation

Library catalogue records as a research resource:introducing 'A Big Data History of Music'

Librarians and archivists are increasingly collecting and working with large quantities of digital data. In science, business, and now the humanities, the production and analysis of vast amounts of data (so-called ‘big data research’) have become fundamental activities. This article introduces the project A Big Data History of Music, a collaboration between Royal Holloway, University of London, and the British Library. The project has made the British Library’s catalogue records for printed and manuscript music available as open data, and has explored how the analysis and visualisation of huge numbers of bibliographic records can open new perspectives for researchers into music history. In addition to the British Library data (over a million records), the project drew on a further million bibliographic descriptions from RISM, which have also recently been released as open data. To show the challenges posed by the heterogeneous nature of the data, the article outlines the different structures of the various catalogue records used in the project, and summarises how the British Library data was cleaned and enhanced prior to its public release. Examples are given of how music-bibliographical data can be analysed and visualised, and how scholars and citizen scientists can engage with this data through hackathons, large-scale data analyses, and database construction. It is hoped this article will encourage other research libraries to consider making their catalogue records available as open data

La diversificación de la oferta turística en las estaciones de media montaña: el caso de la Chapelle en los Alpes franceses del Norte

La Chapelle is a small ski resort in the northern Alps, France. This paper explores the tourist activities offer diversification in mid-altitude mountain areas. The results show that: 1) despite the low altitude ski area (1000-1800 m), the commune’s policy is still heavily in favour of skiing; 2) Summer activities appear to be primarily developed by the ski lift group (mountain biking, devalkart, rollerherbe... thereby creating the need for ski lifts during the summer) rather than local public policy makers. Agritourism, rural and cultural activities appear neglected.Este trabajo estudia la diversificación de la oferta turística en la media montaña (el caso de La Chapelle, pequeña estación de esquí, Alpes del Norte, Francia). 1/ A pesar de la modesta altitud de la zona esquiable (1000-1800 m), La Chapelle apuesta fuertemente por el esquí. 2/ La oferta de verano parece estar más ligada a la organización que administra los telesillas (descenso en bicicletas de montaña, devalkart o rollerherbe que permite rentabilizar los telesillas en verano) que a las políticas públicas locales. El agroturismo, las actividades rurales o culturales no se tienen en cuenta

Najnowsze osiągnięcia turyzmu alpejskiego w aspekcie cyklu rozwoju

The article presents recent trends in the tourist economy in the Alpine region. With reference to the development cycle of the tourist product, proposed by Butler, the author claims that the Alpine tourist centres have already reached the maturity stage. Their further development requires active intervention in order to refresh the tourist product. Further in the article, a variety of tourist development strategies have been presented, which might improve the poor condition of many tourist centres in the region under discussion.W artykule przedstawiono najnowsze trendy w zakresie gospodarki turystycznej w regionie Alp. Autor, w nawiązaniu do przedstawionego przez Butlera cyklu rozwojowego produktu turystycznego, stwierdził, że alpejskie ośrodki turystyczne osiągnęły już etap dojrzałości. Dalszy ich rozwój wymaga podjęcia aktywnej interwencji, zmierzającej do odświeżenia produktu turystycznego. W dalszej części pracy zaprezentowano różne strategie rozwoju turystyki, mające na celu zmianę trudnej sytuacji wielu ośrodków turystycznych w omawianym regionie

Variant load of mitochondrial DNA in single human mesenchymal stem cells

\ua9 The Author(s) 2024.Heteroplasmic mitochondrial DNA (mtDNA) variants accumulate as humans age, particularly in the stem-cell compartments, and are an important contributor to age-related disease. Mitochondrial dysfunction has been observed in osteoporosis and somatic mtDNA pathogenic variants have been observed in animal models of osteoporosis. However, this has never been assessed in the relevant human tissue. Mesenchymal stem cells (MSCs) are the progenitors to many cells of the musculoskeletal system and are critical to skeletal tissues and bone vitality. Investigating mtDNA in MSCs could provide novel insights into the role of mitochondrial dysfunction in osteoporosis. To determine if this is possible, we investigated the landscape of somatic mtDNA variation in MSCs through a combination of fluorescence-activated cell sorting and single-cell next-generation sequencing. Our data show that somatic heteroplasmic variants are present in individual patient-derived MSCs, can reach high heteroplasmic fractions and have the potential to be pathogenic. The identification of somatic heteroplasmic variants in MSCs of patients highlights the potential for mitochondrial dysfunction to contribute to the pathogenesis of osteoporosis

Therapeutic potential of somatic cell nuclear transfer for degenerative disease caused by mitochondrial DNA mutations

Induced pluripotent stem cells (iPSCs) hold much promise in the quest for personalised cell therapies. However, the persistence of founder cell mitochondrial DNA (mtDNA) mutations limits the potential of iPSCs in the development of treatments for mtDNA disease. This problem may be overcome by using oocytes containing healthy mtDNA, to induce somatic cell nuclear reprogramming. However, the extent to which somatic cell mtDNA persists following fusion with human oocytes is unknown. Here we show that human nuclear transfer (NT) embryos contain very low levels of somatic cell mtDNA. In light of a recent report that embryonic stem cells can be derived from human NT embryos, our results highlight the therapeutic potential of NT for mtDNA disease, and underscore the importance of using human oocytes to pursue this goal

A review: Interlayer joining of nickel base alloys

This article provides a comprehensive review of the improvements and results in interlayer bonding of nickel and its alloys to other metal alloys. The development of the interlayer bonding process in joining nickel-based alloys is of high interest to the aerospace and power generation industries, with the idea of on-site repair of turbine components of focus. History of the diffusion bonding process has been summarized, and bonding parameters and methods for various alloy combinations have been outlined. The relationship between hardness and strength, to the intermetallic compounds and porosity present in the bond region has been illustrated. The literature shows the methods for manipulating the volume of these compounds, and subsequent strength. The paper also shows the microstructural changes that occur during interlayer bonding and how these may be manipulated by changing bonding parameters and interlayer composition. Recent and influential papers have been summarized, with the key findings outlined, the type of interlayer and alloy/s being joined have been headlined for ease of navigation, when available, bond strengths and mechanical property values have been highlighted to illustrate bond soundness. This review does not concern traditional fusion welding methods

Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency.

Mutations in several mitochondrial DNA and nuclear genes involved in mitochondrial protein synthesis have recently been reported in combined respiratory chain deficiency, indicating a generalized defect in mitochondrial translation. However, the number of patients with pathogenic mutations is small, implying that nuclear defects of mitochondrial translation are either underdiagnosed or intrauterine lethal. No comprehensive studies have been reported on large cohorts of patients with combined respiratory chain deficiency addressing the role of nuclear genes affecting mitochondrial protein synthesis to date. We investigated a cohort of 52 patients with combined respiratory chain deficiency without causative mitochondrial DNA mutations, rearrangements or depletion, to determine whether a defect in mitochondrial translation defines the pathomechanism of their clinical disease. We followed a combined approach of sequencing known nuclear genes involved in mitochondrial protein synthesis (EFG1, EFTu, EFTs, MRPS16, TRMU), as well as performing in vitro functional studies in 22 patient cell lines. The majority of our patients were children (<15 years), with an early onset of symptoms <1 year of age (65%). The most frequent clinical presentation was mitochondrial encephalomyopathy (63%); however, a number of patients showed cardiomyopathy (33%), isolated myopathy (15%) or hepatopathy (13%). Genomic sequencing revealed compound heterozygous mutations in the mitochondrial transfer ribonucleic acid modifying factor (TRMU) in a single patient only, presenting with early onset, reversible liver disease. No pathogenic mutation was detected in any of the remaining 51 patients in the other genes analysed. In vivo labelling of mitochondrial polypeptides in 22 patient cell lines showed overall (three patients) or selective (four patients) defects of mitochondrial translation. Immunoblotting for mitochondrial proteins revealed decreased steady state levels of proteins in some patients, but normal or increased levels in others, indicating a possible compensatory mechanism. In summary, candidate gene sequencing in this group of patients has a very low detection rate (1/52), although in vivo labelling of mitochondrial translation in 22 patient cell lines indicate that a nuclear defect affecting mitochondrial protein synthesis is responsible for about one-third of combined respiratory chain deficiencies (7/22). In the remaining patients, the impaired respiratory chain activity is most likely the consequence of several different events downstream of mitochondrial translation. Clinical classification of patients with biochemical analysis, genetic testing and, more importantly, in vivo labelling and immunoblotting of mitochondrial proteins show incoherent results, but a systematic review of these data in more patients may reveal underlying mechanisms, and facilitate the identification of novel factors involved in combined respiratory chain deficiency