Managing cultural audiences in a technological society

La diversidad de públicos culturales es un hecho evidente. La diversidad de intereses, gustos y universos personales hace necesaria una relación personalizada con los públicos potenciales de un proyecto cultural para conocerlos mejor, proponerles prácticas culturales de acuerdo con sus intereses y expectativas, para generar confianza recíproca que permita su implicación proactiva en el proyecto en forma de comunidad de intereses.El marketing y las tecnologías digitales nos dan instrumentos y metodologías que no podemos desaprovechar. El marketing relacional y las plataformas CRM nos pueden ayudar a construir relaciones duraderas con los públicos basadas en la confianza que fomenten el empoderamiento y la implicación. Pero hay que elaborar un Plan de Gestión de Públicos que defina prioridades y estrategias de acuerdo con la misión y los objetivos del proyecto. Se proponen cinco estrategias básicas de gestión de públicos a partir de una categorización de los mismos: diversificar, incrementar y regularizar las prácticas de los públicos activos; gestionar la comunidad de públicos implicados; captar nuevos públicos a partir de la demanda latente; invertir en la formación de gustos y valores en niños y adolescentes; crear interés por las prácticas culturales en públicos con demanda inexistente.The diversity of cultural audiences is an obvious fact. The diversity of interests, tastes and personal worlds necessitates a personal relationship with the potential audiences of a cultural project in order to get to know them better, propose cultural practices according to their interests and expectations and build a mutual trust that allows their proactive involvement in a project in the form of a community of interest.Digital technologies and marketing have given us tools and methodologies we must not waste. Relational marketing and CRM platforms can help us forge lasting trust-based relationships with audiences that foster empowerment and involvement. Yet, an Audience Management Plan that defines priorities and strategies in accordance with a project’s mission and objectives must be devised. We propose five basic strategies for managing audiences based on their classification: diversification, improvement and regulation of active audience practices, management of the community of audiences involved; targeting of new audiences on the basis of latent demand; investment in the formation of tastes and values in children and adolescents; and creation of interest in cultural practices in audiences in which no demand exists

El joc tradicional a la iconografia filatèlica

Des del Grup de Recerca i Investigació sobre Esport i Societat (GRIES) ha semblat escaient iniciar un projecte de recerca que permeti aprofundir en l’estudi de la “Cultura lúdica com a patrimoni”. Aquest projecte de recerca i la dedicada a “Esport i modernització a Catalunya en el segle XX” són els dos projectes que sostenen una de les quatre línies d’investigació desplegades per aquest grup de recerca, en concret aquella que fa referència a “Cultura, esport i patrimoni”. Doncs bé, el present article dóna a conèixer els resultats obtinguts en tres estudis en relació a la utilització d’una de les fonts iconogràfiques que al nostre entendre no ha merescut la suficient atenció per part dels investigadors malgrat disposar d’informacions força valuoses, si més no, en l’àmbit de l’estudi dels jocs populars i tradicionals. Ens estem referim a la iconografia filatèlica. El primer dels estudis mostra els resultats obtinguts en la investigació d’una emissió filatèlica que aplegava força manifestacions lúdiques, la “Serie Europa –Juegos infantiles – 1989”. El segon ha consistit en l’elaboració d’un catàleg filatèlic de jocs tradicionals d’Europa i el seu corresponent anàlisi. Per últim, el tercer, pretén consolidar aquest projecte, amb la realització d’una tesi doctoral que pretén estudiar els jocs i esports tradicionals d’arreu del món a través de la filatèlia

Comparative gene expression profiling between human cultured myotubes and skeletal muscle tissue

<p>Abstract</p> <p>Background</p> <p>A high-sensitivity DNA microarray platform requiring nanograms of RNA input facilitates the application of transcriptome analysis to individual skeletal muscle (SM) tissue samples. Culturing myotubes from SM-biopsies enables investigating transcriptional defects and assaying therapeutic strategies. This study compares the transcriptome of aneurally cultured human SM cells versus that of tissue biopsies.</p> <p>Results</p> <p>We used the Illumina expression BeadChips to determine the transcriptomic differences between tissue and cultured SM samples from five individuals. Changes in the expression of several genes were confirmed by QuantiGene Plex assay or reverse transcription real-time PCR. In cultured myotubes compared to the tissue, 1216 genes were regulated: 583 down and 633 up. Gene ontology analysis showed that downregulated genes were mainly associated with cytoplasm, particularly mitochondria, and involved in metabolism and the muscle-system/contraction process. Upregulated genes were predominantly related to cytoplasm, endoplasmic reticulum, and extracellular matrix. The most significantly regulated pathway was mitochondrial dysfunction. Apoptosis genes were also modulated. Among the most downregulated genes detected in this study were genes encoding metabolic proteins AMPD1, PYGM, CPT1B and UCP3, muscle-system proteins TMOD4, MYBPC1, MYOZ1 and XIRP2, the proteolytic CAPN3 and the myogenic regulator MYF6. Coordinated reduced expression of five members of the GIMAP gene family, which form a cluster on chromosome 7, was shown, and the GIMAP4-reduction was validated. Within the most upregulated group were genes encoding senescence/apoptosis-related proteins CDKN1A and KIAA1199 and potential regulatory factors HIF1A, TOP2A and CCDC80.</p> <p>Conclusions</p> <p>Cultured muscle cells display reductive metabolic and muscle-system transcriptome adaptations as observed in muscle atrophy and they activate tissue-remodeling and senescence/apoptosis processes.</p

Descripciones de algunos instrumentos para enseñar a los ciegos las primeras letras y la escritura en notas de música

A portada: Antecede una noticia biográfica del autor escrita por Francisco Campderá y CaminCopia digital. Mataró (Barcelona) : Fundación Iluro, 201

Copper Toxicity Associated With an ATP7A-Related Complex Phenotype

The ATP7A gene encodes a copper transporter whose mutations cause Menkes disease, occipital horn syndrome (OHS), and, less frequently, ATP7A-related distal hereditary motor neuropathy (dHMN). Here we describe a family with OHS caused by a novel mutation in the ATP7A gene, including a patient with a comorbid dHMN that worsened markedly after being treated with copper histidinate.info:eu-repo/semantics/publishedVersio

Gene expression profiling identifies molecular pathways associated with collagen VI deficiency and provides novel therapeutic targets

Ullrich congenital muscular dystrophy (UCMD), caused by collagen VI deficiency, is a common congenital muscular dystrophy. At present, the role of collagen VI in muscle and the mechanism of disease are not fully understood. To address this we have applied microarrays to analyse the transcriptome of UCMD muscle and compare it to healthy muscle and other muscular dystrophies. We identified 389 genes which are differentially regulated in UCMD relative to controls. In addition, there were 718 genes differentially expressed between UCMD and dystrophin deficient muscle. In contrast, only 29 genes were altered relative to other congenital muscular dystrophies. Changes in gene expression were confirmed by real-time PCR. The set of regulated genes was analysed by Gene Ontology, KEGG pathways and Ingenuity Pathway analysis to reveal the molecular functions and gene networks associated with collagen VI defects. The most significantly regulated pathways were those involved in muscle regeneration, extracellular matrix remodelling and inflammation. We characterised the immune response in UCMD biopsies as being mainly mediated via M2 macrophages and the complement pathway indicating that anti-inflammatory treatment may be beneficial to UCMD as for other dystrophies. We studied the immunolocalisation of ECM components and found that biglycan, a collagen VI interacting proteoglycan, was reduced in the basal lamina of UCMD patients. We propose that biglycan reduction is secondary to collagen VI loss and that it may be contributing towards UCMD pathophysiology. Consequently, strategies aimed at over-expressing biglycan and restore the link between the muscle cell surface and the extracellular matrix should be considered

GDF-15 is elevated in children with mitochondrial diseases and is induced by mitochondrial dysfunction

Background We previously described increased levels of growth and differentiation factor 15 (GDF-15) in skeletal muscle and serum of patients with mitochondrial diseases. Here we evaluated GDF-15 as a biomarker for mitochondrial diseases affecting children and compared it to fibroblast-growth factor 21 (FGF-21). To investigate the mechanism of GDF-15 induction in these pathologies we measured its expression and secretion in response to mitochondrial dysfunction. Methods We analysed 59 serum samples from 48 children with mitochondrial disease, 19 samples from children with other neuromuscular diseases and 33 samples from aged-matched healthy children. GDF-15 and FGF-21 circulating levels were determined by ELISA. Results Our results showed that in children with mitochondrial diseases GDF-15 levels were on average increased by 11-fold (mean 4046pg/ml, 1492 SEM) relative to healthy (350, 21) and myopathic (350, 32) controls. The area under the curve for the receiver-operating-characteristic curve for GDF-15 was 0.82 indicating that it has a good discriminatory power. The overall sensitivity and specificity of GDF-15 for a cut-off value of 550pg/mL was 67.8% (54.4%-79.4%) and 92.3% (81.5%-97.9%), respectively. We found that elevated levels of GDF-15 and or FGF-21 correctly identified a larger proportion of patients than elevated lev- els of GDF-15 or FGF-21 alone. GDF-15, as well as FGF-21, mRNA expression and protein secretion, were significantly induced after treatment of myotubes with oligomycin and that levels of expression of both factors significantly correlated. Conclusions Our data indicate that GDF-15 is a valuable serum quantitative biomarker for the diagnosis of mitochondrial diseases in children and that measurement of both GDF-15 and FGF-21 improves the disease detection ability of either factor separately. Finally, we demonstrate for the first time that GDF-15 is produced by skeletal muscle cells in response to mitochon- drial dysfunction and that its levels correlate in vitro with FGF-21 level

Clinical and molecular genetic findings in COLQ-mutant congenital myasthenic syndromes

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Long-term survival in a child with severe encephalopathy, multiple respiratory chain deficiency and GFM1 mutations

BACKGROUND: Mitochondrial diseases due to deficiencies in the mitochondrial oxidative phosphorylation system (OXPHOS) can be associated with nuclear genes involved in mitochondrial translation, causing heterogeneous early onset and often fatal phenotypes. CASE REPORT: The authors describe the clinical features and diagnostic workup of an infant who presented with an early onset severe encephalopathy, spastic-dystonic tetraparesis, failure to thrive, seizures and persistent lactic acidemia. Brain imaging revealed thinning of the corpus callosum and diffuse alteration of white matter signal. Genetic investigation confirmed two novel mutations in the GFM1 gene, encoding the mitochondrial translation elongation factor G1 (mtEFG1), resulting in combined deficiencies of OXPHOS. DISCUSSION: The patient shares multiple clinical, laboratory and radiological similarities with the 11 reported patients with mutations involving this gene, but presents with a stable clinical course without metabolic decompensations, rather than a rapidly progressive fatal course. Defects in GFM1 gene confer high susceptibility to neurologic or hepatic dysfunction and this is, to the best of our knowledge, the first described patient who has survived beyond early childhood. Reporting of such cases is essential so as to delineate the key clinical and neuroradiological features of this disease and provide a more comprehensive view of its prognosis

Transcriptomic profiling of TK2 deficient human skeletal muscle suggests a role for the p53 signalling pathway and identifies growth and differentiation factor-15 as a potential novel biomarker for mitochondrial myopathies

BACKGROUND: Mutations in the gene encoding thymidine kinase 2 (TK2) result in the myopathic form of mitochondrial DNA depletion syndrome which is a mitochondrial encephalomyopathy presenting in children. In order to unveil some of the mechanisms involved in this pathology and to identify potential biomarkers and therapeutic targets we have investigated the gene expression profile of human skeletal muscle deficient for TK2 using cDNA microarrays. RESULTS: We have analysed the whole transcriptome of skeletal muscle from patients with TK2 mutations and compared it to normal muscle and to muscle from patients with other mitochondrial myopathies. We have identified a set of over 700 genes which are differentially expressed in TK2 deficient muscle. Bioinformatics analysis reveals important changes in muscle metabolism, in particular, in glucose and glycogen utilisation, and activation of the starvation response which affects aminoacid and lipid metabolism. We have identified those transcriptional regulators which are likely to be responsible for the observed changes in gene expression. CONCLUSION: Our data point towards the tumor suppressor p53 as the regulator at the centre of a network of genes which are responsible for a coordinated response to TK2 mutations which involves inflammation, activation of muscle cell death by apoptosis and induction of growth and differentiation factor 15 (GDF-15) in muscle and serum. We propose that GDF-15 may represent a potential novel biomarker for mitochondrial dysfunction although further studies are required