Surface Characterisation of Kolk-Boils within Tidal Stream Environments Using UAV Imagery

Funding: This work was funded by the Bryden Centre project, supported by the European Union’s IN- TERREG VA Programme, and managed by the Special EU Programmes Body (SEUPB). The views and opinions expressed in this paper do not necessarily reflect those of the European Commission or SE- UPB. Aspects of this research were also funded by a Royal Society Research Grant [RSG\R1\180430], the NERC VertIBase project [NE/N01765X/1] and the UK Department for Business, Energy and Industrial Strategy’s offshore energy Strategic Environmental Assessment programme. Data Availability Statement The data underlying this article will be shared on reasonable request to the corresponding author. Acknowledgments We gratefully acknowledge the support of Julien Martin, colleagues at Marine Scotland Science and the crew and scientists of the MRV Scotia 2016 and 2018 cruises (particularly Chief Scientists Eric Armstrong and Adrian Tait). We also acknowledge the work contributed by ERI interns: Gael Gelis and Martin Forestier.Peer reviewedPublisher PD

In vitro effect of N-acetyl-L-cysteine on glutathione and sulfhydryl levels in X-linked adrenoleukodystrophy patients

Introduction: Recent evidence shows that oxidative stress seems to be related with the pathophysiology of X-linked adrenoleukodystrophy (X-ALD), a neurodegenerative disorder. Methods: In the present study, the in vitro effect of N-acetyl-L-cysteine (NAC) on glutathione (GSH) and sulfhydryl levels in X-ALD patients was evaluated. Results: A significant reduction of GSH and sulfhydryl content was observed in X-ALD patients compared to the control group. Furthermore, 5 mM of NAC, in vitro, led to an increase in GSH content and sulfhydryl groups in these patients. Conclusion: These data probably indicate that an adjuvant therapy with the antioxidant NAC could improve the oxidative imbalance in X-ALD patients. Keywords: X- linked adrenoleukodystrophy; N-acetyl-L-cysteine; glutathione; sulfhydry

Globotriaosylceramide is correlated with oxidative stress and inflammation in Fabry patients treated with enzyme replacement therapy

AbstractFabry disease is an X-linked inborn error of glycosphingolipid catabolism due to deficient activity of α-galactosidase A that leads to accumulation of the enzyme substrates, mainly globotriaosylceramide (Gb3), in body fluids and lysosomes of many cell types. Some pathophysiology hypotheses are intimately linked to reactive species production and inflammation, but until this moment there is no in vivo study about it. Hence, the aim of this study was to investigate oxidative stress parameters, pro-inflammatory cytokines and Gb3 levels in Fabry patients under treatment with enzyme replacement therapy (ERT) and finally to establish a possible relation between them. We analyzed urine and blood samples of patients under ERT (n=14) and healthy age-matched controls (n=14). Patients presented decreased levels of antioxidant defenses, assessed by reduced glutathione (GSH), glutathione peroxidase (GPx) activity and increased superoxide dismutase/catalase (SOD/CAT) ratio in erythrocytes. Concerning to the damage to biomolecules (lipids and proteins), we found that plasma levels of malondialdehyde (MDA) and protein carbonyl groups and di-tyrosine (di-Tyr) in urine were increased in patients. The pro-inflammatory cytokines IL-6 and TNF-α were also increased in patients. Urinary Gb3 levels were positively correlated with the plasma levels of IL-6, carbonyl groups and MDA. IL-6 levels were directly correlated with di-Tyr and inversely correlated with GPx activity. This data suggest that pro-inflammatory and pro-oxidant states occur, are correlated and seem to be induced by Gb3 in Fabry patients

Efeito in vitro do Óleo de Lorenzo sobre alguns parâmetros de estresse oxidativo em córtex e fígado de ratos jovens

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Avaliação de parâmetros de estresse oxidativo na adrenoleucodistrofia

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