Multifaceted superoxide dismutase 1 expression in amyotrophic lateral sclerosis patients: a rare occurrence?

Amyotrophic lateral sclerosis (ALS) is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex, brainstem, and spinal cord. While the typical clinical phenotype of ALS involves both upper and lower motor neurons, human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions, expanding the phenotype of ALS. Although superoxide dismutase 1 (SOD1) mutations represent a minority of ALS cases, the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized therapies. Despite numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1 (SOD1-ALS), no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this subpopulation. In this narrative review, we analyze and discuss the available literature on extrapyramidal and non-motor features during SOD1-ALS. The multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms, pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS

Protocells Either Synchronize or Starve

Two different processes take place in self-reproducing protocells, i.e., (i) cell reproduction by fission and (ii) duplication of the genetic material. One major problem is indeed that of assuring that the two processes take place at the same pace, i.e., that they synchronize, which is a necessary condition for sustainable growth. In previous theoretical works, using dynamical models, we had shown that such synchronization can spontaneously emerge, generation after generation, under a broad set of hypotheses about the architecture of the protocell, the nature of the self-replicating molecules, and the types of kinetic equations. However, an important class of cases (quadratic or higher-order self-replication) did not synchronize in the models we had used, but could actually lead to divergence of the concentration of replicators. We show here that this behavior is due to a simplification of the previous models, i.e., the “buffering” hypothesis, which assumes instantaneous equilibrium of the internal and external concentrations of those compounds which can cross the cell membrane. That divergence disappears if we make use of more realistic dynamical models, with finite transmembrane diffusion rates of the precursors of replicators

Political elections and market reactions: the ‘Trump effect’ on green stocks

The election of Donald Trump as the 45th president of the United States and his sceptical positions on climate threaten the fight against climate change, potentially weakening investors’ green concerns. Through an event study approach, we aim to analyse the reaction of the U.S. stock market to the latest presidential election, exploring the investors’ reactions across sectors. We find a strong heterogeneous reaction across sectors. Moreover, we show that the worst performance in the short period is attributable to companies with better performance on environmental issues, which could mean an adjustment of investors’ assessment criteria in anticipation of President Trump's anti-climate policies, reduced transition risk for "brown" firms and lower benefits for firms excelling in environmental performance

Citizenship, Math and Gender: Exploring Immigrant Students' Choice of Majors

This study examines the impact of host-country citizenship on immigrant students' choice of academic majors, using data from an Italian university and incorporating characteristics of students' countries of origin. The analysis focuses on enrolment in fields of study categorized by mathematical content. The findings reveal three main points: First, obtaining citizenship reduces the likelihood of choosing math-related disciplines; second, this effect is more pronounced among women, further widening the gender gap in math-intensive fields; and third, these gaps are larger among students from more gender-equal countries but are less affected by the acquisition of citizenship. These results are supported by matching techniques, two-stage least squares, and robustness and sensitivity analyses. Given that math-intensive fields are linked to higher earning potential, the findings suggest that investment in mathematical skills may serve as a safeguard against labour market risks—a necessity that lessens upon acquiring citizenship, especially for women. Although this shift could adversely affect future earnings, it also contributes to a more even distribution of students across disciplines, potentially enhancing diversity in occupations where immigrants are traditionally under-represented

L'entità dell'enfisema nei pazienti con CPFE e IPF: significato prognostico e implicazioni cliniche per l'approccio terapeutico

La Sindrome Fibrosi-Enfisema Combinati (CPFE) è una pneumopatia interstiziale fumo-relata a prognosi sfavorevole caratterizzata dalla coesistenza di enfisema e fibrosi, con volumi relativamente preservati per l'equilibrio tra restrizione e ostruzione polmonare. Sebbene la coesistenza dell’enfisema influenzi significativamente la sopravvivenza dei pazienti con fibrosi, i dati sulla significatività della sua estensione e sugli esiti clinici sono scarsi. La terapia broncodilatatrice inalatoria ha dimostrato efficacia nel migliorare la qualità di vita (QoL) in pazienti con pattern ostruttivo, tuttavia, mancano dati del suo effetto nei pazienti con CPFE. Questo progetto di ricerca mira a studiare l'impatto clinico dell'enfisema (E) nei pazienti con fibrosi polmonare per quanto riguarda la prognosi e la risposta alla terapia con broncodilatatori. Il primo progetto è stato condotto mediante uno studio retrospettivo, osservazionale, longitudinale che ha raccolto dati (epidemiologia, comorbilità, funzionalità polmonare e mortalità) di 155 pazienti dell'Ospedale Universitario di Bellvitge (Barcellona) alla diagnosi,1, 3, 5 anni: 43 CPFE(>10%E), 31 IPF+E (5% e 10%E), 31 IPF senza E e 50 BPCO-E. Lo studio ha dimostrato che i pazienti con CPFE e IPF+E avevano maggiore esposizione al fumo e prevalenza di ipertensione polmonare (PH), cancro polmonare, E parasettale e pneumotorace. I pazienti con CPFE presentavano maggiore dispnea e fabbisogno di ossigeno (O2) alla diagnosi, con progressione più elevata durante il primo anno (valutata con distanza e O2-terapia al 6MWT). I pazienti avevano una sopravvivenza peggiore proporzionale all'entità dell'enfisema e ancora peggiore nella CPFE-IPF (CPFE-IPF >IPF+E >IPF senza E;p<0,001). Alla diagnosi i pazienti con CPFE rispetto ai pazienti con IPF presentavano valori più elevati di FVC, RV, FRC, che aumentavano gradualmente con l'estensione dell'enfisema; valori più bassi di MEF50%,25% (flusso espiratorio massimo), FEV1 e KCO (coefficiente di trasferimento di CO) che diminuivano gradualmente con l'estensione dell'enfisema. I pazienti con CPFE hanno avuto maggiore prevalenza di comorbidità e un tasso di progressione più elevato durante il primo anno dopo la diagnosi. Lo studio ha rilevato che la mortalità dei pazienti con CPFE-IPF e IPF e le comorbidità erano proporzionali all’entità dell’enfisema. Il secondo progetto è stato condotto con uno studio retrospettivo, osservazionale, longitudinale, multicentrico includendo i pazienti dello studio precedente e pazienti con CPFE e IPF+E afferenti al Centro per le Malattie Rare del Polmone dell'Ospedale Universitario di Modena (MaRP), per un totale di 90 pazienti con CPFE e 35 con IPF+E. Sono stati valutati come possibili fattori prognostici indici spirometrici come il declino di FEV1+FVC in 1 anno> del 5 o 10%. Il terzo progetto è stato condotto mediante uno studio prospettico monocentrico che ha arruolato 30 pazienti con CPFE senza deficit ostruttivo riferiti al Centro MaRP di Modena con l'obiettivo di valutare variazioni su sintomi, QoL, funzione respiratoria e prestazione funzionale in seguito a terapia broncodilatatrice per 3 mesi. Sintomi, QoL (mediante SGRQ, mMRC, CAT), volumi polmonari mediante test di funzionalità polmonare (PFT) e prestazioni funzionali mediante 6MWT sono stati misurati al basale e a 3 mesi. Al basale i punteggi medi QoL erano 33(23-46) per SGRQ, 2(2-3) per mMRC, 15(11-21) per CAT. A 3 mesi è stato registrato un calo significativo dei 3 punteggi (SGRQ -10,9, mMRC -0,82, CAT -4,9,p<0,0001). Inoltre sono state misurate una riduzione significativa del RV (p=0,048) e tendenza all'aumento di distanza al 6MWT (p=0,07). Questo studio ha dimostrato l'efficacia della terapia con broncodilatatori su QoL e volume residuo nei pazienti con CPFE.Combined Pulmonary Fibrosis and Emphysema Syndrome (CPFE) is a smoking-related interstitial lung disease with poor prognosis, characterized by the coexistence of emphysema and fibrosis, with relatively preserved lung volumes due to the balance of restriction and obstruction. Although the coexistence of emphysema significantly affects the survival of patients with concurrent pulmonary fibrosis, data on the significance of its extent and clinical outcomes are scarce. Inhaled bronchodilator therapy has shown effectiveness in improving quality of life (QoL) in patients with obstructive patterns. However, data on the effects of these drugs in patients with CPFE are lacking. This research project aims to study the clinical impact of emphysema (E) in patients with pulmonary fibrosis regarding prognosis and response to bronchodilator therapy. The first project was conducted with a retrospective, observational and longitudinal study that collected data (epidemiology, comorbidities, pulmonary function, mortality) from 155 patients of Bellvitge University Hospital (Barcelona) at diagnosis, 1, 3, 5 years: 43 CPFE (>10% E), 31 IPF with E (5% and 10%E), 31 IPF without E, and 50 COPD with E. The study showed that patients with CPFE and IPF with E had greater exposure to smoking and a higher prevalence of pulmonary hypertension (PH), lung cancer, paraseptal E and pneumothorax. Patients with CPFE exhibited greater dyspnea and oxygen (O2) need at diagnosis, with more rapid progression during the first year after diagnosis (assessed based on distance and O2 therapy in the 6MWT). The survival rate was worse and proportional to the extent of E, with even poorer outcomes in CPFE-IPF (CPFE-IPF > IPF with E > IPF without E;p<0.001). At diagnosis patients with CPFE compared to those with IPF had higher values of FVC, RV, FRC, which gradually increased with the extent of emphysema; and lower values of MEF50%, 25% (maximum expiratory flow), FEV1 and KCO (CO transfer coefficient) that decreased gradually with the extent of emphysema. Patients with CPFE had a higher prevalence of comorbidities and a greater rate of progression during the first year after diagnosis. The study found that mortality in patients with CPFE-IPF and IPF and comorbidities were directly proportional to the extent of emphysema. The second project was conducted as a retrospective, observational, and longitudinal multicenter study, including patients from the previous study and patients with CPFE and IPF+E referring to the Center of Rare Lung Disease of University Hospital of Modena (MaRP). In total 90 patients with CPFE and 35 with IPF+E were evaluated. Potential prognostic factors were assessed, focusing on spirometric indicators such as a decline in FEV1+FVC over 1 year greater than 5% or 10%. The third project was conducted with a monocentric prospective study that enrolled 30 patients with CPFE with no obstruction referred to the MaRP of Modena, aimed at evaluating changes in symptoms, QoL, respiratory function, and functional performance following bronchodilator therapy for 3 consecutive months. Symptoms, QoL (measured by SGRQ, mMRC, CAT), pulmonary volumes through pulmonary function tests (PFT), and functional performance with the 6-minute walk test (6MWT) were assessed at baseline and after 3 months. At baseline the average QoL scores were 33 (23-46) for SGRQ, 2 (2-3) for mMRC, 15 (11-21) for CAT. At 3 months, a significant decrease in all 3 scores was recorded (SGRQ -10.9, mMRC -0.82, and CAT -4.9, p<0.0001). Additionally a significant reduction in RV was measured (p=0.048) and there was a trend towards increased distance in the 6MWT (p=0.07). This study demonstrated the effectiveness of bronchodilator therapy on QoL and residual volume in patients with CPFE

International networks in cultural industries: the case of agencies for opera artists in the immediate post-COVID-19 period

This study presents an empirical investigation into the internationalization processes within the opera performing arts industry, which represents a critical component of the broader creative industries. By utilizing data from Operabase, a comprehensive online database of opera-related information, we employ social network analysis to delineate the network of connections established between nations through the mobility of opera artists, mediated by the agencies that represent them. Moreover, the research assesses the influence of factors such as geographical proximity, cultural affinity, market dominance, and reciprocity on these international networks. Our findings provide a nuanced understanding of the internationalization of opera, emphasizing the significance of geographical proximity, reciprocity, and (partially) tradition/dominance in the opera market as key drivers of global interconnectedness in this domain

Der (un)gute Freund in Moskau. Argumentative Strukturen der kriegsbedingten Neupositionierung eines populistischen Vorbilds (in corso di stampa)

Before the outbreak of the war, both Italian and German populist parties regarded Vladimir Putin, sometimes explicitly and at other times less overtly, as a political role model whose leadership style and decisions served as a source of inspiration for their respective national governments. The aim of this paper is to investigate how the linguistic portrayal of the Russian president has changed in the discourses of leading politicians from populist right-wing parties in Italy and Germany as a result of the war-related developments. The analysis is based on transcripts of oral statements, primarily from public speeches and appearances on talk shows, made by the top representatives of these parties. The focus is on whether linguistic positioning strategies can be identified that depict Putin as a ›friend‹ before the invasion of Ukraine and possibly as a ›foe‹ after the war began. Particularly relevant is the question of whether the politicians’ linguistic portrayals reveal signs of embarrassment or discomfort in light of the war of aggression initiated by Putin