No abstract

ObjectivesSpinocerebellar ataxia type 3/Machado–Joseph disease (SCA3/MJD) is a polyglutamine disorder with no current disease-modifying treatment. Conformational changes in mutant ataxin-3 trigger different pathogenic cascades, including reactive oxygen species (ROS) generation; however, the clinical relevance of oxidative stress elements as peripheral biomarkers of SCA3/MJD remains unknown. We aimed to evaluate ROS production and antioxidant defense capacity in symptomatic and presymptomatic SCA3/MJD individuals and correlate these markers with clinical and molecular data with the goal of assessing their properties as disease biomarkers.MethodsMolecularly confirmed SCA3/MJD carriers and controls were included in an exploratory case–control study. Serum ROS, measured by 2′,7′-dichlorofluorescein diacetate (DCFH-DA) as well as superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) antioxidant enzyme activities, levels were assessed.ResultsFifty-eight early/moderate stage symptomatic SCA3/MJD, 12 presymptomatic SCA3/MJD, and 47 control individuals were assessed. The DCFH-DA levels in the symptomatic group were 152.82 nmol/mg of protein [95% confidence interval (CI), 82.57–223.08, p < 0.001] higher than in the control and 243.80 nmol/mg of protein (95% CI, 130.64–356.96, p < 0.001) higher than in the presymptomatic group. The SOD activity in the symptomatic group was 3 U/mg of protein (95% CI, 0.015–6.00, p = 0.048) lower than in the presymptomatic group. The GSH-Px activity in the symptomatic group was 13.96 U/mg of protein (95% CI, 5.90–22.03, p < 0.001) lower than in the control group and 20.52 U/mg of protein (95% CI, 6.79–34.24, p < 0.001) lower than in the presymptomatic group and was inversely correlated with the neurological examination score for spinocerebellar ataxias (R = −0.309, p = 0.049).ConclusionEarly/moderate stage SCA3/MJD patients presented a decreased antioxidant capacity and increased ROS generation. GSH-Px activity was the most promising oxidative stress disease biomarker in SCA3/MJD. Further longitudinal studies are necessary to identify both the roles of redox parameters in SCA3/MJD pathophysiology and as surrogate outcomes for clinical trials.

No abstract

Craft beers are known for their distinct flavor, brew, and regional distribution. They are made using top-fermenting (ale) yeast, bottom-fermenting (lager) yeast, or through spontaneous fermentation. Craft beers are consumed and produced in Brazil in large quantities. However, they present a high level of polyphenols, which affects consumer preference as they may yield a taste of bitterness to beers. In this study, we analyzed the relationship between polyphenols and bitterness as well as the composition of the main styles of craft beers and consumer preference for them. Six different styles were analyzed according to their polyphenol content, bitterness, chemical composition, sensory profile, and preference. For preference, a panel of 62 untrained assessors was used. For sensory profile, quantitative descriptive analysis was performed using expert assessors (n = 8). The most preferred style was classic American pilsner, and the least preferred was standard American lager. The most preferred style showed less bitterness (9.52) and lower polyphenol content (0.61 mg EAG/mL), total solids (6.75 °Brix), and turbidity (7.27 NTU). This beer also exhibited reduced sensory notes of malty, fruity, smoked, hoppy, and phenolic but a higher perception of floral, sweet, and yeast notes; the bitterness attribute had a reduced perception. This study advances the understanding and complexity of the sensory profile of different styles of craft beers from Southern Brazil.