36 research outputs found

    Quantum-well-laser mirror degradation investigated by microprobe optical spectroscopy

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    A study of facet degradation of InGaAs quantum well lasers is reported. We tune up a Raman and photoluminescence micro-probe technique for determining the crystal structure and the temperature profile of the cladding layer, in steps of approximately 1 micrometer, with a temperature resolution better than 1 degree Kelvin. The cladding layer composition and cross- section temperature profile have been monitored during operation. A clear correlation between the facet degradation and the type of protective coating is found

    Aromatic L-amino acid decarboxylase deficiency: a patient-derived neuronal model for precision therapies

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    Aromatic L-amino acid decarboxylase (AADC) deficiency is a complex inherited neurological disorder of monoamine synthesis which results in dopamine and serotonin deficiency. The majority of affected individuals have variable, though often severe cognitive and motor delay, with a complex movement disorder and high risk of premature mortality. For most, standard pharmacological treatment provides only limited clinical benefit. Promising gene therapy approaches are emerging, though may not be either suitable or easily accessible for all patients. In order to better characterize the underlying disease pathophysiology and guide precision therapies, we generated a patient-derived midbrain dopaminergic (mDA) neuronal model of AADC deficiency from induced pluripotent stem cells (iPSCs). The neuronal model recapitulates key disease features, including absent AADC enzyme activity and dysregulated dopamine metabolism. We observed developmental defects affecting synaptic maturation and neuronal electrical properties, which were improved by lentiviral gene therapy. Bioinformatic and biochemical analyses on recombinant AADC predicted that the activity of one variant could be improved by L-3,4-dihydroxyphenylalanine (L-DOPA) administration; this hypothesis was corroborated in the patient-derived neuronal model, where L-DOPA treatment leads to amelioration of dopamine metabolites. Our study has shown that patient-derived disease modelling provides further insight into the neurodevelopmental sequelae of AADC deficiency, as well as a robust platform to investigate and develop personalised therapeutic approaches

    Quantum-well-laser mirror degradation investigated by microprobe optical spectroscopy

    Get PDF
    A study of facet degradation of InGaAs quantum well lasers is reported. We tune up a Raman and photoluminescence micro-probe technique for determining the crystal structure and the temperature profile of the cladding layer, in steps of approximately 1 micrometer, with a temperature resolution better than 1 degree Kelvin. The cladding layer composition and cross- section temperature profile have been monitored during operation. A clear correlation between the facet degradation and the type of protective coating is found

    Aromatic l-amino acid decarboxylase deficiency: a patient-derived neuronal model for precision therapies

    Get PDF
    Aromatic l-amino acid decarboxylase (AADC) deficiency is a complex inherited neurological disorder of monoamine synthesis which results in dopamine and serotonin deficiency. The majority of affected individuals have variable, though often severe cognitive and motor delay, with a complex movement disorder and high risk of premature mortality. For most, standard pharmacological treatment provides only limited clinical benefit. Promising gene therapy approaches are emerging, though may not be either suitable or easily accessible for all patients. To characterize the underlying disease pathophysiology and guide precision therapies, we generated a patient-derived midbrain dopaminergic neuronal model of AADC deficiency from induced pluripotent stem cells. The neuronal model recapitulates key disease features, including absent AADC enzyme activity and dysregulated dopamine metabolism. We observed developmental defects affecting synaptic maturation and neuronal electrical properties, which were improved by lentiviral gene therapy. Bioinformatic and biochemical analyses on recombinant AADC predicted that the activity of one variant could be improved by l-3,4-dihydroxyphenylalanine (l-DOPA) administration; this hypothesis was corroborated in the patient-derived neuronal model, where l-DOPA treatment leads to amelioration of dopamine metabolites. Our study has shown that patient-derived disease modelling provides further insight into the neurodevelopmental sequelae of AADC deficiency, as well as a robust platform to investigate and develop personalized therapeutic approaches

    LCCA and TCO: a How-to Approach to Assess the Costs in the Customer’s Eye

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    The servitization process and in particular Product-Service Systems offering, and the growing attention to issues of economic, environmental and social sustainability, lead both companies and customers to evaluate the solution offered along the life-cycle taking into account all the economic and/or environmental costs. To this aim, methodologies such as Life-Cycle Costing, Life-Cycle Assessment, and Total Cost of Ownership are extremely helpful. This paper, through multiple case studies, aims to present different ways of collaborations among companies, universities, research centers, certification bodies etc., through which tools, methods, human resources, data, information, can be exploited to develop TCO models for different purposes
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