171 research outputs found

    Quantification of the Chemical Chaperone 4-Phenylbutyric Acid (4-PBA) in Cell Culture Media via LC-HRMS: Applications in Fields of Neurodegeneration and Cancer

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    In recent years, 4-phenylbutyric acid (4-PBA), an FDA-approved drug, has increasingly been used as a nonspecific chemical chaperone in vitro and in vitro, but its pharmacodynamics is still not clear. In this context, we developed and validated a Liquid Chromatography–High Resolution Mass Spectrometry (LC-HRMS) method to quantify 4-PBA in NeuroBasal-A and Dulbecco’s Modified Eagle widely used cell culture media. Samples were injected on a LunaÂź 3 ”m PFP(2) 100 Å (100 × 2.0 mm) column maintained at 40 °C. Water and methanol both with 0.1% formic acid served as mobile phases in a step gradient mode. The mass acquisition was performed by selected ion monitoring (SIM) in negative mode for a total run time of 10.5 min at a flow rate of 0.300 mL/min. The analogue 4-(4-Nitrophenyl)-Butyric Acid served as internal standard. Validation parameters were verified according to FDA and EMA guidelines. The quantification ranges from 0.38–24 ”M. Inter and intraday RSDs (Relative Standard Deviations) were within 15%. The developed LC-HRMS method allowed the estimation of 4-PBA absorption and adsorption kinetics in vitro in two experimental systems: (i) 4-PBA improvement of protein synthesis in an Alzheimer’s disease astrocytic cell model; and (ii) 4-PBA reduction of endoplasmic reticulum stress in thapsigargin-treated melanoma cell lines. © 2023 by the authors

    A review of the MSCA ITN ECOSTORE - Novel complex metal hydrides for efficient and compact storage of renewable energy as hydrogen and electricity

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    Hydrogen as an energy carrier is very versatile in energy storage applications. Developments in novel, sustainable technologies towards a CO2-free society are needed and the exploration of all-solid-state batteries (ASSBs) as well as solid-state hydrogen storage applications based on metal hydrides can provide solutions for such technologies. However, there are still many technical challenges for both hydrogen storage material and ASSBs related to designing low-cost materials with low-environmental impact. The current materials considered for all-solid-state batteries should have high conductivities for Na+, Mg2+ and Ca2+, while Al3+-based compounds are often marginalised due to the lack of suitable electrode and electrolyte materials. In hydrogen storage materials, the sluggish kinetic behaviour of solid-state hydride materials is one of the key constraints that limit their practical uses. Therefore, it is necessary to overcome the kinetic issues of hydride materials before discussing and considering them on the system level. This review summarizes the achievements of the Marie SkƂodowska-Curie Actions (MSCA) innovative training network (ITN) ECOSTORE, the aim of which was the investigation of different aspects of (complex) metal hydride materials. Advances in battery and hydrogen storage materials for the efficient and compact storage of renewable energy production are discussed

    Metal Hydrides and Related Materials - Energy Carriers for Novel Hydrogen and Electrochemical Storage

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    The seventh edition of the International Renewable and Sustainable Energy Conference (IRSEC) was held in Agadir (Sofitel Royal Bay, November 27–30, Morocco) under the Program Chair of Prof. Ahmed Ennaoui (IRESEN). IRSEC, as one of the biggest conferences in north Africa, aims at creating an international forum to facilitate discussions and exchanges in all aspects of renewable and sustainable energy. This Viewpoint will summarize the scientific presentations and stimulated discussions during the Special Session (November 28–29) on Metal Hydrides’ Energy covering topics of metal hydrides and energy related issues for innovative processes and technologies, with a focus on magnesium-based hydrides, intermetallic hydrides, complex and melt hydrides, porous materials, and thin films

    Fatigue, reduced sleep quality and restless legs syndrome in Charcot-Marie-Tooth disease: a web-based survey

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    To investigate the prevalence of fatigue, daytime sleepiness, reduced sleep quality, and restless legs syndrome (RLS) in a large cohort of patients with Charcot-Marie-Tooth disease (CMT) and their impact on health-related quality of life (HRQoL). Participants of a web-based survey answered the Epworth Sleepiness Scale, the Pittsburgh Sleep Quality Index, the Multidimensional Fatigue Inventory, and, if the diagnostic criteria of RLS were met, the International RLS Severity Scale. Diagnosis of RLS was affirmed in screen-positive patients by means of a standardized telephone interview. HRQoL was assessed by using the SF-36 questionnaire. Age- and sex-matched control subjects were recruited from waiting relatives of surgical outpatients. 227 adult self-reported CMT patients answered the above questionnaires, 42.9% were male, and 57.1% were female. Age ranged from 18 to 78 years. Compared to controls (n = 234), CMT patients reported significantly higher fatigue, a higher extent and prevalence of daytime sleepiness and worse sleep quality. Prevalence of RLS was 18.1% in CMT patients and 5.6% in controls (p = 0.001). RLS severity was correlated with worse sleep quality and reduced HRQoL. Women with CMT were affected more often and more severely by RLS than male patients. With regard to fatigue, sleep quality, daytime sleepiness, RLS prevalence, RLS severity, and HRQoL, we did not find significant differences between genetically distinct subtypes of CMT. HRQoL is reduced in CMT patients which may be due to fatigue, sleep-related symptoms, and RLS in particular. Since causative treatment for CMT is not available, sleep-related symptoms should be recognized and treated in order to improve quality of life

    Smart cities in a smart world

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    Very often the concept of smart city is strongly related to the flourishing of mobile applications, stressing the technological aspects and a top-down approach of high-tech centralized control systems capable of resolving all the urban issues, completely forgetting the essence of a city with its connected problems. The real challenge in future years will be a huge increase in the urban population and the changes this will produce in energy and resource consumption. It is fundamental to manage this phenomenon with clever approaches in order to guarantee a better management of resources and their sustainable access to present and future generations. This chapter develops some considerations on these aspects, trying to insert the technological issues within a framework closer to planning and with attention to the social impact
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