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This study suggests that moderate daily intake of PHGG can contribute to relieving depressive-like behavior.

This study suggests that moderate daily intake of PHGG can contribute to relieving depressive-like behavior.

Pyruvate kinase deficiency (PKD) is a rare, autosomal recessive red blood cell enzyme disorder, which leads to lifelong hemolytic anemia and associated complications from the disease and its management.

An international, multicenter registry enrolled 124 individuals younger than 18years old with molecularly confirmed PKD from 29 centers. EED226 Retrospective and prospective clinical data were collected.

There was a wide range in the age at diagnosis from 0 to 16years. Presentation in the newborn period ranged from asymptomatic to neonatal jaundice to fulminant presentations of fetal distress, myocardial depression, and/or liver failure. Children <5years old were significantly more likely to be transfused than children >12 to <18years (53% vs. 14%, p=.0006), which correlated with the timing of splenectomy. Regular transfusions were most common in children with two severe PKLR variants. In regularly transfused children, the nadir hemoglobin goal varied considerably. Impact on quality of life was a common reason for treatment with regular blood transfusions and splenectomy. Splenectomy increased the hemoglobin and decreased transfusion burden in most children but was associated with infection or sepsis (12%) and thrombosis (1.3%) even during childhood. Complication rates were high, including iron overload (48%), perinatal complications (31%), and gallstones (20%).

There is a high burden of disease in children with PKD, with wide practice variation in monitoring and treatment. Clinicians must recognize the spectrum of the manifestations of PKD for early diagnostic testing, close monitoring, and management to avoid serious complications in childhood.

There is a high burden of disease in children with PKD, with wide practice variation in monitoring and treatment. Clinicians must recognize the spectrum of the manifestations of PKD for early diagnostic testing, close monitoring, and management to avoid serious complications in childhood.Aiming at the construction of novel platform for efficient light harvesting, the precise synthesis of a new family of AIEgen-branched rotaxane dendrimers was successful realized from an AIEgen-functionalized [2]rotaxane through a controllable divergent approach. In the resultant AIE macromolecules, up to twenty-one AIEgens located at the tails of each branches, thus making them the first successful example of AIEgen-branched dendrimers. Attributed to the solvent-induced switching feature of the rotaxane branches, the integrated rotaxane dendrimers displayed interesting dynamic feature upon the aggregation-induced emission (AIE) process. Moreover, novel artificial light-harvesting systems were further constructed based on these AIEgen-branched rotaxane dendrimers, which revealed impressive generation-dependent photocatalytic performances for both photooxidation reaction and aerobic cross-dehydrogenative coupling (CDC) reaction.Oxygen-sensing mechanisms have evolved to allow organisms to respond and adapt to oxygen availability. In metazoans, oxygen-sensing is predominantly mediated by the hypoxia inducible factors (HIFs). These transcription factors are stabilised when oxygen is limiting, activating genes involved in angiogenesis, cell growth, pH regulation and metabolism to reset cell function and adapt to the cellular environment. However, the recognition that other cellular pathways and enzymes can also respond to changes in oxygen abundance provides further complexity. Dissecting this interplay of oxygen-sensing mechanisms has been a key research goal. Here, we review how genetic approaches have contributed to our knowledge of oxygen-sensing pathways which to date have been predominantly focused on the HIF pathway. We discuss how genetic studies have advanced the field and outline the implications and limitations of such approaches for the development of therapies targeting oxygen-sensing mechanisms in human disease.Essential oils (EOs) are commercially important products, sources of compounds with antioxidant and antimicrobial activities considered indispensable for several fields, such as the food industry, cosmetics, perfumes, pharmaceuticals, sanitary and agricultural industries. In this context, this systematic review and meta-analysis, a novel approach will be presented using chemometric tools to verify and recognize patterns of antioxidant, antibacterial, and antifungal activities of EOs according to their geographic, botanical, chemical, and microbiological distribution. Scientific papers were selected following the Preferred Reporting Items for Systematic Review and Meta-Analyses statement flow diagram, and the data were evaluated by the self-organizing map and hierarchical cluster analysis. Overall, this novel approach allowed us to draw an overview of antioxidants and antimicrobials activities of EOs reported in 2019, through 585 articles evaluated, obtaining a dataset with more than 10,000 data, distributed in more than 80 countries, 290 plant genera, 150 chemical compounds, 30 genera of bacteria, and 10 genera of fungi. The networks for geographic, botanical, chemical, and microbiological distribution indicated that Brazil, Asia, the botanical genus Thymus, species Thymus vulgaris L. “thyme,” the Lamiaceae family, limonene, and the oxygenated monoterpene class were the most representative in the dataset, while the species Escherichia coli and Candida albicans were the most used to assess the antimicrobial activity of EOs. This work can be seen as a guide for the processing of metadata using a novel approach with non-conventional statistical methods. However, this preliminary approach with EOs can be extended to other sources or areas of food science.Vertebrate pancreas organogenesis is a stepwise process regulated by a complex network of signaling and transcriptional events, progressively steering the early endoderm toward pancreatic fate. Many crucial players of this process have been identified, including signaling pathways, cis-regulatory elements, and transcription factors (TFs). Pancreas-associated transcription factor 1a (PTF1A) is one such TF, crucial for pancreas development. PTF1A mutations result in dramatic pancreatic phenotypes associated with severe complications, such as neonatal diabetes and impaired food digestion due to exocrine pancreatic insufficiency. Here, we present a brief overview of vertebrate pancreas development, centered on Ptf1a function and transcriptional regulation, covering similarities and divergences in three broadly studied organisms human, mouse and zebrafish.