Mucopolysaccharidosis IVA: Diagnosis, Treatment, and Management.

Mucopolysaccharidosis type IVA (MPS IVA, or Morquio syndrome type A) is an inherited metabolic lysosomal disease caused by the deficiency of the N-acetylglucosamine-6-sulfate sulfatase enzyme. The deficiency of this enzyme accumulates the specific glycosaminoglycans (GAG), keratan sulfate, and chondroitin-6-sulfate mainly in bone, cartilage, and its extracellular matrix. GAG accumulation in these lesions leads to unique skeletal dysplasia in MPS IVA patients. Clinical, radiographic, and biochemical tests are needed to complete the diagnosis of MPS IVA since some clinical characteristics in MPS IVA are overlapped with other disorders. Early and accurate diagnosis is vital to optimizing patient management, which provides a better quality of life and prolonged life-time in MPS IVA patients. Currently, enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT) are available for patients with MPS IVA. However, ERT and HSCT do not have enough impact on bone and cartilage lesions in patients with MPS IVA. Penetrating the deficient enzyme into an avascular lesion remains an unmet challenge, and several innovative therapies are under development in a preclinical study. In this review article, we comprehensively describe the current diagnosis, treatment, and management for MPS IVA. We also illustrate developing future therapies focused on the improvement of skeletal dysplasia in MPS IVA

Los olores como factor de disconfort en ambientes laborales interiores

En el presente trabajo se analiza la problemática derivada de emisiones y olores en instalaciones y centros de trabajo situados en el interior de los edificios, partiendo de los últimos avances que en este campo se han desarrollado para su control en ambientes exteriores. Para ello se describen las bases científicas en que se apoya la teoría del olfato. Se efectúa una sucinta revisión de la normativa vigente promulgada por las administraciones implicadas, así como de la normativa técnica que le afecta. Por último se expone la propuesta de un procedimiento operativo que recoge las quejas y sugerencias suscitadas en ambientes laborales por motivos de olor y la manera de gestionarlas

Innovations for sustainability in the roll-out of the Sustainable Development Goals

Companies have been adapting their strategic decisions in order to align with Sustainable Development Goals since 2015. A motivation for companies to align their strategic decisions with Sustainable Development Goals is to gain legitimacy among supranational organizations, governments, and civil society. Some demonstrate the strength of their commitment to these goals by investing in innovations designed to boost their organizational performance; while others turn to greenwashing in a bid to maintain profits. Investing in sustainability innovations has become a key manifestation of firms’ commitment to Sustainable Development. This study aims to analyse the interaction between sustainability commitment, innovations for sustainability and organizational performance. A sample of 3,420 companies for the period 2015 to 2020 is used to test two working hypotheses. Despite the significant gains it brings in terms of sustainability performance, the results show that investing in innovation for sustainability carries the risk of short-term losses. This has several implications. Some companies may subscribe to Sustainable Development Goals in their pursuit of legitimacy rather than out of true commitment. However, actual engagement in innovation for sustainability can attract potential investors, and, in our view, should be encouraged by politicians and lawmakers

Crítica de Libros.

Sin resumen disponible. solo texto completo

Trehalose accumulation induced during the oxidative stress response is independent of TPS1 mRNA levels in Candida albicans

Growing cells of the Candida albicans trehalose- deficient mutant tps1/tps1 were extremely sensitive to severe oxidative stress exposure (H2O2). However, their viability was not affected after saline stress or heatshock treatments, being roughly equivalent to that of the parental strain. In wild-type cells, these adverse conditions induced the intracellular accumulation of trehalose together with activation of trehalose-6P synthase, whereas the endogenous trehalose content and the corresponding biosynthetic activity were barely detectable in the tps1/tps1 mutant. The addition of cycloheximide did not prevent the marked induction of trehalose-6P synthase activity. Furthermore, the presence of H2O2 decreased the level of TPS1 mRNA expression. Hence, the conspicuous trehalose accumulation in response to oxidative stress is not induced by increased transcription of TPS1. Our results are consistent with a specific requirement of trehalose in order to withstand a severe oxidative stress in C. albicans, and suggest that trehalose accumulation observed under these conditions is a complex process that most probably involves post-translational modifications of the trehalose synthase complex

Energy Recovery in Capacitive Deionization Technology

Capacitive deionization technique (CDI) represents an interesting alternative to compete with reverse osmosis by reducing energy consumption. It is based on creating an electric field between two electrodes to retain the salt ions on the electrode surface by electrostatic attraction; thus the CDI cell operates as a supercapacitor storing energy during the desalination process. Most of the CDI research is oriented to improving the electrode materials in order to increase the effective surface and ionic retention. However, if the CDI overall efficiency is to be improved, it is necessary to optimize the CDI cell geometry and the charge/discharge current used during the deionization process. A DC/DC converter is required to transfer the stored energy from one cell to another with the maximum possible efficiency during energy recovery, thus allowing the desalination process to continue. A detailed description of energy losses and the DC/DC converter used to recover part of the energy involved in the CDI process will provide the hints to optimize the efficiency of the CDI technique for water desalination. The proposed chapter presents an electric model to characterize the power losses in CDI cells and the power converter required for the energy recovery process