Search

This article extends the literature on consumers' responses to green claims and their intentions to buy green products. We used an experimental approach based on three complementary studies. The first compares the effects of two claims regarding different levels of a company's environmental commitment. The second examines whether consumers can recognize exaggerated claims. The third analyzes consumers' perceptions of a company's environmental commitment and intentions to buy the advertised product when an institutional methodology is used to substantiate the message. Surveys were administered online in January 2020 to samples of the Italian population aged 18 to 75. The results suggest that consumers positively perceive the commitment that lies behind all the conditions tested, strongly influencing intentions to buy. However, consumers have either little or no understanding of differences among green claims and are also easily deceived by exaggerated claims (such as "zero impact"). Using multigroup structural equation modeling (SEM), we highlight the important role of education. Consumers with a higher level of education are more receptive to claims that imply a higher commitment and appreciate the presence of an institutional methodology as a credibility signal. However, even highly educated consumers are likely to be deceived by exaggerated claims. [ABSTRACT FROM AUTHOR]

In the present work, a large database comprising a set of features related to geometry, type of boundary condition, type of material and experimental value of the first natural frequency for 184 historic masonry towers is collected. The database is then used to develop empirical formulas for the estimation of the fundamental frequency related to the first bending mode. The data are divided into a training set and a validation set for a correct assessment of the empirical equations, generated through power regression and artificial neural network. Different combinations of features are exploited as independent variables to investigate their influence on the dynamic behaviour of masonry towers. To improve the accuracy of the prediction, distinct models are generated for specific clusters with likely homogeneous behaviour, according to the boundary conditions (isolated or bounded) and type of materials (brick, stone or mixed masonry). [ABSTRACT FROM AUTHOR]

The growing interest in the circular economy poses a pivotal challenge for the tourism sector. A measurement framework is crucial for guiding the actors of the tourism sector in the development of a long-term strategy for a circular transition. Based on action research, where researchers and practitioners collaborate with each other, a co-created measurement framework was developed based on four types of capital and five circular economy principles. An assessment tool based on 54 Key Performance Indicators (KPIs) was then developed and tested in nine tourism destinations in the Mediterranean region. The framework was further fine-tuned through expert feedback and practitioners support, leading to the final Circular Measurement System for tourism destinations. This represents a solid and stakeholder-based measurement system to assess the circularity of tourism destinations, tangibly promoting the transition of the sector towards a circular economy. [ABSTRACT FROM AUTHOR]

Drawing on legitimacy theory, we study the nexus between green communication and the implementation of green practices, and in particular, we focus on the determinants of their discrepancy. Based on a large sample of firms in 58 countries over a 19‐year period, we employ an index to measure the discrepancy between green operations and the communicated practices, mapped to each firm's board structure. The results provide the first empirical evidence that larger, more gender‐diverse, and more independent boards are associated with a preponderance of green communication over implementation. We interpret this imbalance as a strategy to participate in the public discourse to gain moral legitimacy. Conversely, CEO duality is associated with a discrepancy in the opposite direction, with firms focusing more on implementing green practices than talking about them, suggesting that these firms aim mainly at gaining pragmatic legitimacy from their stakeholders. [ABSTRACT FROM AUTHOR]

Mucopolysaccharidosis type IIIA (MPS IIIA or Sanfilippo syndrome type A) is an autosomal recessive lysosomal storage disorder caused by pathogenic variants in the SGSH gene encoding N‐sulfoglucosamine sulfohydrolase, an enzyme involved in the degradation of heparan sulfate. MPS IIIA is typically characterized by neurocognitive decline and hepatosplenomegaly with childhood onset. Here, we report on a 53‐year‐old male subject initially diagnosed with Usher syndrome for the concurrence of retinitis pigmentosa and sensorineural hearing loss. Clinical exome sequencing identified biallelic missense variants in SGSH, and biochemical assays showed complete deficiency of sulfamidase activity and increased urinary glycosaminoglycan excretion. Reverse phenotyping revealed left ventricle pseudo‐hypertrophy, hepatosplenomegaly, bilateral deep white matter hyperintensities upon brain MRI, and decreased cortical metabolic activity by PET‐CT. On neuropsychological testing, the proband presented only partial and isolated verbal memory deficits. This case illustrates the power of unbiased, comprehensive genetic testing for the diagnosis of challenging mild or atypical forms of MPS IIIA. [ABSTRACT FROM AUTHOR]

Autosomal recessive Stargardt disease (STGD1, MIM 248200) is caused by mutations in the ABCA4 gene. Complete sequencing of ABCA4 in STGD patients identifies compound heterozygous or homozygous disease-associated alleles in 65-70% of patients and only one mutation in 15-20% of patients. This study was designed to find the missing disease-causing ABCA4 variation by a combination of next-generation sequencing (NGS), array-Comparative Genome Hybridization (aCGH) screening, familial segregation and in silico analyses. The entire 140 kb ABCA4 genomic locus was sequenced in 114 STGD patients with one known ABCA4 exonic mutation revealing, on average, 200 intronic variants per sample. Filtering of these data resulted in 141 candidates for new mutations. Two variants were detected in four samples, two in three samples, and 20 variants in two samples, the remaining 117 new variants were detected only once. Multimodal analysis suggested 12 new likely pathogenic intronic ABCA4 variants, some of which were specific to (isolated) ethnic groups. No copy number variation (large deletions and insertions) was detected in any patient suggesting that it is a very rare event in the ABCA4 locus. Many variants were excluded since they were not conserved in non-human primates, were frequent in African populations and, therefore, represented ancestral, and not disease-associated, variants. The sequence variability in the ABCA4 locus is extensive and the non-coding sequences do not harbor frequent mutations in STGD patients of European-American descent. Defining disease-associated alleles in the ABCA4 locus requires exceptionally well characterized large cohorts and extensive analyses by a combination of various approaches.