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Microalgae are able to produce many valuable biomolecules, such as polysaccharides, that presents a large diversity of biochemical structures and functions as antioxidant, antifungal, anticancer, among others. Static magnetic fields (SMF) influence the metabolism of microorganisms and has been shown as an alternative to increase microalgae biomass, yield and compounds production. Especially, some studies have highlighted that SMF application could enhance carbohydrate content. This study aimed to evaluate different conditions of SMF on Spirulina and Chlorella in indoor and outdoor conditions, in order to confirm the influence of SMF on polysaccharides production, evaluating which polysaccharidic fraction could be enhanced by SMF and highlighting a possible modification in EPS composition. Starch from Chlorella and exopolysaccharides (EPS) from Spirulina were quantified and characterized. SMF increased the starch content in Chorella fusca biomass. EPS productions from A. platensis and Spirulina sp. were not significantly increased, and global composition appeared similar to the controls (constituted basically of 80–86% neutral sugars and 13–19% uronic acids). However, the monosaccharide composition analysis revealed a significant modification of composition, i.e., the amount of fucose, arabinose, rhamnose, galactose and glucuronic acid was increased, while the glucose content was decreased. SMF application led to significant modification of polysaccharides production and this study demonstrate that combining the outdoor conditions with SMF, the starch content and EPS composition was positively affected.

Double Skin Façades are complex fenestration systems capable to control solar heat gain and ventilation in buildings. Due to the high flexibility of such innovative components, having energy models able to replicate the thermal behaviour of the Double Skin Facades is of utmost importance for their optimal control and integration with building automation strategies. In this context, a numerical model has been developed and validate within the experimental data. The methodological steps are presented in this work and in the last section, the potential applications of the model are discussed.

Aims and background:Various genes have been identified for monogenic disorders resembling Parkinson’s disease. The products of some of these genes are associated with mitochondria and have been implicated in cellular protection against oxidative damage. In the present study we analysed fibroblasts from a patient carrying the homozygous mutation p.W437X in the PTEN-induced kinase 1 (PINK1), which manifested a very early onset parkinsonism.Results:Patient’s fibroblasts did not show variation in the mtDNA copy number or in the expression of the oxidative phosphorylation complexes. Sequence analysis of the patient’s mtDNA presented two new missense mutations in the ND5 (m.12397A>G, p.T21A) and ND6 (m. 14319T>C, p.N119D) genes coding for two subunits of complex I. The two mutations were homoplasmic in both the patient and the patient’s mother. Patient’s fibroblasts resulted in enhanced constitutive production of the superoxide anion radical that was abrogated by inhibitor of the complex I. Moreover enzyme kinetic analysis of the NADH:ubiquinone oxidoreductase showed changes in the substrates affinity.Conclusion:To our knowledge, this is the first report showing co-segregation of a Parkinson’s disease related nuclear gene mutation with mtDNA mutation(s). Our observation might shed light on the clinical heterogeneity of the hereditary cases of Parkinson’s disease, highlighting the hitherto unappreciated impact of coexisting mtDNA mutations in determining the development and the clinical course of the disease.

Left ventricular hypertrophy (LVH) is a frequent finding in Friedreich’s ataxia (FRDA). In previous studies treatment with idebenone, a synthetic analogue of coenzyme Q10, has been associated with a substantial decrease in myocardial hypertrophy, despite great variability in cardiac responsiveness among patients. Here we present the results of a retrospective analysis of a cohort of 35 patients (20 with LVH, 15 without LVH) with confirmed molecular diagnosis of FRDA, treated with idebenone 5 mg/kg/day for up to five years. At the end of the study, we found an increase of interventricular septum and posterior wall thickness in the group without LVH before treatment and no change in the group with LVH before treatment. The neurological picture of the disease significantly deteriorated with time in both groups.

This study focuses on the control of movable Venetian blinds. Multiple improvements to an existing on/off open-loop control strategy in a case-study apartment have been simulated in TRNSYS 18, thanks to the detailed optical and thermal modelling allowed by the Bidirectional Scattering Distribution Function (BSDF) used as input to the Type56_CFS. The control strategy improvements include the combination of rule-based, closed-loop and discrete state control, in addition to four control strategy activation methods (three use a schedule, and one measures the external temperature). Simulated control inputs include internal temperature, external temperature and vertical irradiance. The results show reductions in overheating, achieved without completely blocking natural illumination or compromising heating demand. While on/off control in winter often leads to increased heating energy consumption, the space sees regular overheating when on/off control is inactive over winter. Conversely, discrete state control is able to more precisely control solar gains in winter to maintain an adequate temperature without utilising the heating system, all the while allowing some level of natural illumination. Ultimately, it is concluded that the choice of the control strategy depends on which objective (minimisation of heating energy consumption, maximisation of daylight harvesting, reduction of overheating risk, etc.) is prioritised.

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disease due to mutations in SACS, which encodes sacsin, a protein localized on the mitochondrial surface and possibly involved in mitochondrial dynamics. In view of the possible mitochondrial involvement of sacsin, we investigated mitochondrial activity at functional and molecular level in skin fibroblasts obtained from ARSACS patients. We observed remarkable bioenergetic damage in ARSACS cells, as indicated by reduced basal, adenosine triphosphate (ATP)-linked and maximal mitochondrial respiration rate, and by reduced respiratory chain activities and mitochondrial ATP synthesis. These phenomena were associated with increased reactive oxygen species production and oxidative nuclear DNA damage. Our results suggest that loss of sacsin is associated with oxidative stress and mitochondrial dysfunction, and thus highlight a novel mechanism in the pathogenesis of ARSACS. The involvement of mitochondria and oxidative stress in disease pathogenesis has been described in a number of other neurodegenerative diseases. Therefore, on the basis of our findings, which suggest a potential therapeutic role for antioxidant agents, ARSACS seems to fall within a larger group of disorders.

The maintenance of railway axles requires an optimization of their inspection intervals in order to reduce costs. Such components can endure more than 30 years of service life and are subjected to scheduled Non Destructive Testing (NDT) inspections. It is therefore important to assess the influence of the severe in-service loads on the propagation of cracks in the axles. This manuscript reports deterministic fracture calculations, for the simulation of cracks propagating through a railway axle, by the Dual Boundary Element Method. Once Stress Intensity Factors along the crack front are obtained, the residual fatigue life estimates were calculated through a user made spreadsheet, taking into account the complex loading conditions of the axles. The presented results support the application of the crack propagation simulation for the determination of axle NDT intervals under different loading conditions.

The study of the role of glycemia in the causation of cardiovascular disease has been limited by several factors, above all by its measurement over time. Non enzymatic glycated proteins in the blood, the product of the non enzymatic reaction of a reducing sugar with the reactive amino acid of a target protein, are an integrated measure of blood glucose over days to weeks. They have been used in the management of clinical diabetes mellitus, but are still infrequently used in epidemiological studies in non diabetic subjects. There are few epidemiological studies that show that glycated hemoglobin, fructosamine, an index of total non enzymatic glycated proteins in the blood, and glycated apolipoprotein B and other non enzymatic glycated proteins in the blood in non diabetic subjects are associated with cardiovascular diseases. In this paper we review: 1) the overall mechanisms of non enzymatic glycation of proteins; 2) the measurement of glycated hemoglobin, fructosamine, and glycated apolipoprotein B and their relationship with blood glucose levels in non diabetic subjects; 3) the association of glycated hemoglobin, fructosamine and glycated apolipoprotein B with cardiovascular disease. We conclude that non enzymatic glycation of protein in the blood is associated with cardiovascular disease also in non diabetic subjects, and could be used to define dietary risk factors of cardiovascular disease.

Objective:Neuropathological descriptions of the brain in Friedreich’s ataxia (FRDA) were obtained before availability of the current molecular genetic tests for this disease. Voxel-based morphometry (VBM) enables an unbiased whole-brain quantitative analysis of differences in gray matter (GM) and white matter (WM) volume.Methods:Using VBM, we assessed the brain structural damage in 22 patients with genetically confirmed FRDA and 25 healthy controls. The results were correlated with the disease duration and the severity of the patients’ clinical deficits—evaluated using the International Cerebellar Ataxia Rating Scale and Inherited Ataxia Clinical Rating Scale.Results:In patients with FRDA, VBM showed a symmetrical volume loss in dorsal medulla, infero-medial portions of the cerebellar hemispheres, the rostral vermis and in the dentate region. No volume loss in cerebral hemispheres was observed. The atrophy of the cerebellum and medulla correlated with the severity of the clinical deficit and disease duration.Conclusions:In patients with FRDA, significant GM and WM loss was observed only in the cerebellum and dorsal medulla. These structural changes correlate with the severity of the clinical deficit and disease duration.

OBJECTIVE To verify if GAA expansion size in Friedreich’s ataxia could account for the severity of sensory neuropathy. METHODS Retrospective study of 56 patients with Friedreich’s ataxia selected according to homozygosity for GAA expansion and availability of electrophysiological findings. Orthodromic sensory conduction velocity in the median nerve was available in all patients and that of the tibial nerve in 46 of them. Data of sural nerve biopsy and of a morphometric analysis were available in 12 of the selected patients. The sensory action potential amplitude at the wrist (wSAP) and at the medial malleolus (m mal SAP) and the percentage of myelinated fibres with diameter larger than 7, 9, and 11 μm in the sural nerve were correlated with disease duration and GAA expansion size on the shorter (GAA1) and larger (GAA2) expanded allele in each pair. Pearson’s correlation test and stepwise multiple regression were used for statistical analysis. RESULTS A significant inverse correlation between GAA1 size and wSAP, m mal SAP, and percentage of myelinated fibres was found. Stepwise multiple regression showed that GAA1 size significantly affects electrophysiological and morphometric data, whereas duration of disease has no effect. Conclusion—The data suggest that the severity of the sensory neuropathy is probably genetically determined and that it is not progressive